United States Department of Agriculture Agricultural Research Service National Agricultural Library Animal Welfare Information Center

Bovine Spongiform Encephalopathy (BSE) and Other Animal Related Transmissible Spongiform Encephalopathies March 2001 (Revised January 2004) AWIC Series #2001-01 Updates Bovine Spongiform Encephalopathy, 1990 Jean Larson United States Department of Agriculture Agricultural Research Service National Agricultural Library Animal Welfare Information Center 10301 Baltimore Avenue Beltsville, MD 20705-2351 Contact us: http://www.nal.usda.gov/awic/contact.php Policies and Links Information on how to request materials that are included in the collection of the National Agricultural Library (NAL) may be found on the Collection Services website at http://www.nal.usda.gov/services/request.shtml. Please read carefully as there are certain restrictions on media and document types.

BSE, also called bovine spongiform encephalopathy or "mad cow disease" is a progressive neurological disorder of cattle that results from infection by an unconventional and extraordinary transmissible agent. BSE is one of several known animal transmissible spongiform encephalopathies including transmissible mink encephalopathy, scrapie, chronic wasting disease of mule deer, and elk, and feline spongiform encephalopathy. The disease name refers to the fact that at the end of the disease, the brain is full of holes like a sponge. The disease may develop in a relatively short time or, as is more usual, will take decades to develop. Scrapie may be the most well known of the spongiform encephalopathies. It occurs in sheep and goats. In general, as in cattle, diseased animals lose coordination of their legs and body movements and eventually cannot stand. The name "scrapie" refers to the fact that the animals can become irritable and develop an intense itch. The unrelenting itch leads them to "scrape" off their wool/fur. Although the majority of the information in this resource is on BSE, the publication covers the recent information on all these diseases as they may affect human health, farm animals and wildlife species.

There are also human conditions that are similar to the animal diseases. In most cases the human diseases are not due to transmissible agents. They can be genetic diseases that run in families, a mutation that happens sporadically in individuals and probably animals as well, or they may be transmitted by ingestion of the infectious agent (e.g. kuru of the Fore people was caused by ritualized cannibalism).

There is still some controversy regarding the nature of the transmissible agent that causes these fatal conditions, but the most accepted theory is that the agent is a modified form of a normal cell surface component known as a prion (proteinaceous infectious articles and (pronounced preeon) protein) (PrP). This modified version of PrP is disease causing, and is both less soluble and more resistant to enzyme degradation then the normal protein. "Currently there is no known treatment for prion diseases, and the fear that prions passed from cattle to humans may be justified." ¹

Dr. Prusiner also states that "prions appear to multiply in an incredible way: they convert normal protein molecules into dangerous ones (PrPsc) simply by inducing the benign molecules to change their shape." "There are hints that the prions causing the diseases " such as BSE and scrapie "may not be the only ones. Prions made of different proteins may contribute to other neurogenerative diseases that are quite prevalent in humans."

It is the transmissible possibilities of the infectious agent moving between animal species and between animals and humans via an oral route that is currently of greatest concern. This concern is due to an outbreak of the BSE disease in United Kingdom (UK) cattle, and the increase of a human spongiform encephalopathy Creutzfeldt-Jakob Disease in the British population that was exposed to meat from cattle that had BSE.

BSE was first recognized in Great Britain in November 1986. The first cases probably occurred in early 1985. It is not definitive that the disease originated from scrapie infected meat and bone meal that was used as a protein supplement in cattle feeds, but there is strong evidence and general agreement that the outbreak was amplified by feeding rendered infected cattle meat-and-bone meal to young calves. "Some other captive ungulates, captive exotic cats and domesticated cats in the UK contracted the disease probably by eating the same feed material. During the peak of the disease (1992), about 1% of the adult cattle in the UK had the disease. Lower incidences have occurred in indigenous cattle in Ireland, France, Switzerland, the Netherlands, and Portugal. A few cases have been recorded in Canada, Germany, Denmark, Italy, the Falkland Islands, and the Sultanate of Oman in animals exported from Great Britain." ²

As of November 2000, in more than 35,000 herds, about 177,500 cases of BSE were confirmed in the UK alone. For current information, see the website of the Office of International Des Epizooties at http://www.oie.int/eng/info/en_esb.htm. According to the Animal and Plant Health Inspection Service of the U.S. Department of Agriculture, the first case of BSE in the United States was confirmed on December 23, 2003. The USDA's web site provides current news and surveillance information on BSE in the United States.

Concurrent with the cattle epidemic in the UK has been a rise of a new variant of CJD (NvCJD) in humans. This form of CJD "predominately affects younger individuals (median age at death 27.5 years as of October 2000), has atypical clinical features, coordination problems within weeks or months, dementia and myoclonus late in the illness, a duration of illness of at least 6 months, and an abnormal brain scan. There is beginning to be strong epidemiologic and laboratory evidence for a causal relationship between the NvCJD and BSE. The absence of confirmed cases of NvCJD in other geographic areas free of BSE supports a causal relationship." ³

Because of the BSE and NvCJD incidence, the Animal Welfare Information Center decided to provide a science based information resource about these diseases and all other potentially important TSEs. This resource includes a bibliographic listing of articles and website resources about the disease, the emergence of the disease in the UK and the political and social events surrounding the BSE crisis. It is a dynamic resource and additional materials on BSE and other TSEs will be added as they are identified.

References:

1. Prusiner, S.B. Prion biology and diseases fatal conformations of proteins during a journey from heresy to orthodoxy. In Prions and Brain Diseases in Animals and Humans. Edited by D.R.O. Morrison. Plenum Press, NY 1998, p. 135-139. 30 refs. ISBN 0-306-45825-X. Part of the NATO ASI series. Series A, Life Sciences: v. 295. It is the proceeding of a NATO Advanced Research workshop on Prions and Brain Diseases in Animals and Humans, held August 19-23, 1996, in Erice, Italy.

2. The Merck Veterinary Manual 8th Edition. eds. S.E. Aiello and A. Mays. Published by Merck & Co., Inc. of Whitehouse Station, NJ. and in cooperation with Merial Limited. Printed by National Publishing Inc. of Philadelphia, PA 1998, p. 897. ISBN: 0-911910-29-8

3. Center for Disease Control, National Center for Infectious Diseases. Questions and Answers Regarding Bovine Spongiform Encephalopathy (BSE) and Creutzfeldt-Jakob Disease. Bovine Spongiform Encephalopathy and Creutzfeldt-Jakob Disease. April, 2001 http://www.cdc.gov/ncidod/dvrd/vcjd/qa.htm

2004

1. Garcia, Rebeca; Jukes, David. The Spanish system of food controls: Its administration and enforcement. Food Control. January 2004; 15(1): 51-59. ISSN: 0956-7135
   NAL call no.: TP372.7.F66
   Descriptors: food safety, BSE, dioxin, European Food Safety Authority (EFSA), EU, Spanish Agency of Food Safety, general structure, Spanish ministries, Spain.

2. Liechti, R. The international conference on bovine spongiform encephalopathy and food safety, April 17-18, 2002. Food Control. January 2004; 15(1): 71-77. ISSN: 0956-7135
   NAL call no.: TP372.7.F66
   Descriptors: current scientific knowledge, BSE, TSEs, food safety risks, a forum for discussion, prion diseases, social science, consumer groups.

3. Moya, K.L.; Hassig, R.; Creminon, C.; Laffont, Di Giamberardino, L. Enhance and retrograde axonal transport of Prpc in peripheral nerve. Journal of Neurochemistry. 2004; 88(1): 155-160. ISSN: 0022-3042.
   NAL call no.: QP351.J6
   Descriptors: PrPC axonal transport, abundance of PrPC in peripheral nerves, prion protein resistant to detergent extraction, prion neuroinvastion, nerve extraction conditions, prion disease application, TSE entry pathway, prion neuroinvasion, scrapie, CNS access.

2003

1. Adjou, Karim Tarik; Simoneau, Steve; Sales, Nicole; Lamoury, Francois; Dormont, Dominique; Papy-Garcia, Dulce; Barritault, Denis; Deslys, Jean Philippe; Lasmezas, Corinne Ida. A novel generation of heparan sulfate mimetics for the treatment of prion diseases.  Journal of General Virology. September 2003; 84(9): 2595-2603.  ISSN:  0022-1317
   NAL call no.:  QR360.A1J6
   Descriptors: transmission of spongiform encephalopathies, PrPres, protease resistant abnormal form, cellular prion protein, heparan sulfate mimetics, HM 2602, effect is to abolish prion propagation in scrapie-infected GT1 cells, in vivo testing, 263K scrapie-infected hamsters, toxicity, dextran sulfate 500, mode of action. 

2. Alexandru, N. Detectia PrPSC in trunchiul cerebral la doua ovine in Romania.  [Detection of PrPSC in the brainstem of two ewes in Romania.]  Revista Romana de Medicina Veterinara. 2003; 13(1): 75-81.  ISSN:  1220-3173. In Romanian with an English summary. 
   NAL call no.:  SF604.R48
   Descriptors: ewes, sheep, natural scrapie diagnosed, prion diseases, brain stem samples by foramen magnum rapid method, immunohistochemistry, Romania. 

3. Belay, Ermias D.; Maddox, Ryan A.; Gambetti, Pierluigi; Schonberger, Lawrence B. Monitoring the occurrence of emerging forms of Creutzfeldt-Jakob disease in the United States.  Neurology. January 28, 2003; 60(2): 176-181. ISSN:  0028-3878
   Descriptors: BSE, 19 European countries, Israel, Japan, vCJD in four European countries, U.S. Centers for Disease Control, National Prion Disease Pathology Surveillance Center, human cases.

4. Bhakdi, S.; Bohl, J. Prions and mad cow disease. Kraftfutter. 2003, 86(3): 56-65. ISSN:  0023-4427.  In English and German.
   NAL call no.:  389.78 K85
   Descriptors: BSE, cattle, cows, bovine spongiform encephalopathy, CJD, epidemiology, prion disease, foodborne meat diseases, new infections declining in UK.

5. Bozzetta, E.; Caramelli, M.; Casalone, C.; Acutis, P.L.; Ru, G. BSE surveillance in Italy: Neuropathological findings in cattle in the frame of the passive surveillance programme.  Journal of Veterinary Medicine, Series A. February 2003; 50(1): 48-49. ISSN: 0931-184X
   NAL call no.:  41.8 Z4
   Descriptors: cattle, BSE, the 272/98/EC Decision, 52 animals with clinical signs, CNS, histopathology, immunohistochemistry (IHC) or Western blot (WB) for PrPSc, no BSE detected, improvement in passive surveillance needed. 

6. Bosques, Carlos J.; Imperiali, Barbara. The interplay of glycosylation and disulfide formation influences fibrillization in a prion protein fragment.  Proceedings of the National Academy of Sciences of the United States of America. June 24, 2003; 100(13): 7593-7598. ISSN:  0027-8424
   NAL call no.:  500 N21P
   Descriptors: prion proteins, PrPC, preteinase K-resistant prion protein scrapie, PrPSc, role of glycosylation, disulfide stability, retarding rate of fibril formation, intermolecular disulfide formation via Cys-179, structure transition. 

7. Brodmann, Peter D.; Moor, Dominik. Sensitive and semi-quantitative TaqManTM real-time polymerase chain reaction systems for the detection of beef (Bos taurus) and the detection of the family Mammalia in food and feed. Meat Science. September 2003; 65(1): 599-607. ISSN: 0309-1740
   NAL call no.:  TX373.M4
   Descriptors: beef, BSE contamination bovine-based products,  vCJD, variant Creutzfeld Jacob Disease, consumer confidence, PCR analysis, mammal DNA, beef DNA, meat and bone meal products, two methods.

8. Brown, David R. Prion protein expression modulates neuronal copper content.  Journal of Neurochemistry. October 2003; 87(2): 377-385.  ISSN:  0022-3042
   NAL call no.:  QP351.J6
   Descriptors: prion protein, copper binding capacity, prion disease, transmissible spongiform encephalopathies, copper content of the brain, transgenic mice animal model, copper levels of the synapse, age effects.

9. Brown, D.A.; Bruce, M.E.; Fraser, J.R. Comparison of the neuropathological characteristics of bovine spongiform encephalopathy (BSE) and variant Creutzfeldt-Jakob disease (vCJD) in mice. Neuropathology and Applied Neurobiology. June 2003; 29(3): 262-272.  ISSN: 0305-1846
   Descriptors: neuropathology, inbred mouse strains, RIII, C57BL, VM, C57BL x VM, PrPres deposition, astrocytosis, vacuolation, vCJD linked to BSE, comparison study.    

10. Bruce, Moira E. TSE strain variation. British Medical Bulletin. 2003; 66: 99-108.  ISSN:  0007-1420
    Descriptors: scrapie, cattle, goats, humans, mice, prion disease, strain differences, transmissible spongiform encephalopathy.

11. Concepcion, G. P.; Padlan, E.A. Are humans getting 'mad-cow disease' from eating beef, or something else? Medical Hypotheses.  May 2003; 60(5): 699-701. ISSN:  0306-9877
    Descriptors: hypothesis, gastric digestion of human and various animal prion proteins, ingestion of infected rodent parts, possibly droppings, possible transmission mode of scrapie or BSE to humans.

12. Carp, Richard I.; Kascsak, Richard J. Taking aim at the diagnosis of TSE infectious agents.  Abstracts of Papers American Chemical Society. 2003; 226(1-2): ANYL 9.  ISSN:  0065-7727.  Note:  The 226th American Chemical Society National Meeting, New York, NY, USA, September 07-11, 2003
    NAL call no.:  381 Am33Pa
    Descriptors: BSE, bovine spongiform encephalopathy, diagnosis, prion-disease, transmissible spongiform encephalopathy, variant Creutzfeldt-Jakob disease, vCJD.  

13. Castilla, J.; Gutierrez, Adan A.; Brun, A.; Pintado, B.; Ramirez, M.A.; Parra, B.; Doyle, D.; Rogers, M.; Salguero, F.J.; Sanchez, C.; Sanchez-Vizcaino, J.M.; Torres, J.M. Early detection of PrPres in BSE-infected bovine PrP transgenic mice.  Archives of Virology.  April 2003; 148(4): 677-691. ISSN:  0304-8608
    NAL call no.:  448.3 AR23
    Descriptors: BSE, scrapie, transgenic mouse lines, bovine protein gene expression at different levels, lines exhibited characteristics of bovine disease, PrPres detected, Western blot, immunohistochemistry assays, prions levels of inoculum, pathognomonic markers of disease, incubation period, prion changes from original infection. 

14. Chaala, A.; Roy, C. Recycling of meat and bone meal animal feed by vacuum pyrolysis.  Environmental Science and Technology. October 1, 2003; 37(19): 4517-4522.  ISSN:  0013-936X
    NAL call no.:  TD420 A1E5
    Descriptors: BSE, European beef, alternative disposal of waste animal products and bone meal, vacuum pyrolysis, laboratory reactor, animal flour, combustible gas, high calorific value oil, mineral residue, aqueous phase of organic, pollution control/reduction.

15. Caughey, Byron. Prion protein conversions: Insight into mechanisms, TSE transmission barriers and strains. British Medical Bulletin. 2003; 66: 109-120.  ISSN: 0007-1420
    Descriptors: prions, transmissible spongiform encephalopathies, disease transmission factors, prion protein isoforms, biochemistry, scrapie.

16. Chesebro, Bruce. Introduction to the transmissible spongiform encephalopathies or prion diseases.  British Medical Bulletin.  2003; 66: 1-20.  ISSN:  0007-1420
    Descriptors: TSE, BSE, CJD, prion diseases, etiology, prion protein, disease transmission, theories. 

17. Clauss, M. Do cows fed BSE-infected meat and bone meal in the colostrum-producing stage pass on infectious BSE agent to their calves?  Medical Hypotheses. October 2003; 61(4): 439-443. ISSN:  0306-9877
    Descriptors: BSE, cows, calves, disease transmission, digestion of infectious agent in meat and bone meal, colostrum infectivity, hypothesis.  

18. Cranmer, Morris; McChesney, Thomas. Chronic wasting disease: Risks to hunters and consumers of deer and elk meat.  Neurotoxicology.  March 2003; 24(2): 313-314. ISSN:  0161-813X.  Note: Twentieth International Neurotoxicology Conference:  Emerging Issues in Neurotoxicology, Little Rock, AR, USA, November 18-21, 2002.
    NAL call no.:  RC321.N437
    Descriptors: elk, deer, chronic wasting disease, human health risks, prion disease, transmissible spongiform encephalopathy, meat product, abstract.

19. Dahlanuddin; Van, Tien Dam; Liang, J.B.; Adams, D.B. An exploration of risk factors for bovine spongiform encephalopathy in ruminant production systems in the tropics.  Revue Scientifique et Technique Office International des Epizooties.  April 2003; 22(1): 271-281. ISSN:  0253-1933
    NAL call no.:  SF781.R4
    Descriptors: world risk of BSE, cattle, ruminant production systems, presence of infective agent, transmission and amplification of disease, lack of cattle-based meat and bone meal reduces risks, South East Asia.    

20. Dahms, S. Epidemiologische Modellbildung am Beispiel BSE -- Betrachtungen aus statistischer Sicht. [Epidemiological modelling taking BSE as an example -- reflections from a statistical viewpoint.  Berliner und Munchener Tierarztliche Wochenschrift. 2003, 116(1-2): 22-30.  ISSN: 0005-9366.  In German.
    NAL call no.:  41.8 B45
    Descriptors: BSE, epidemiology modeling, analytical methods, epidemiological surveys, epidemiology, mathematical models, nervous system diseases.  

21. Daude, Nathalie; Marella, Mathieu; Chabry, Joelle. Specific inhibition of pathological prion protein accumulation by small interfering RNAs.  Journal of Cell Science. July 1, 2003; 116(13): 2775-2779. ISSN:  0021-9533
    NAL call no.:  QH301.J6
    Descriptors: transmissible spongiform encephalopathies (TSEs) pathogenesis, PrP, prion protein, proteinase-K resistant isoform, PrPres, small interfering RNA (siRNA) duplexestrigger specific Prnp gene silencing, scrapie-infected neuroblastoma cells, possible therapeutic approach for treatment of prion disease.

22. De Vlieger, J.J.; Puister-Jansen, L.F.; Sengers, H.H.W.J.M.; Ouweltjes, W. Ontwikkelingen in de export van Nederlands fokvee. [Developments in the export of Dutch breeding cattle.]  Rapport, Landbouw Economisch Instituut LEI. 2003; No. 2.03.07, 60 pp.  ISBN:  90-5242-803-4.  In Dutch.
    Descriptors: dairy cattle, Holstein-Friesian, effects of BSE and FMD on markets, animal welfare, European Union, exports, international trade, laws, legislation, market competition, prion diseases, transport of animals, viral diseases, Algeria, Denmark, France, Germany, Lebanon, Netherlands, Poland, Spain.

23. Dedet, V. En tysk ante mortem BSE blodprove vurderes: Forelobige resultater praesenteret pa World Buiatric 2002.  [A German ante-mortem BSE blood test is evaluated. Provisional results presented at the World Buiatric conference, 2002.]  Dansk Veterinaertidsskrift. 2003; 86(2): 24.  ISSN:  0106-6854.  In Danish.
    NAL call no.:  41.9 D23
    Descriptors: cattle, BSE, bovine spongiform encephalopathy, ante-mortem blood test, diagnostic technique, short-strand RNA coated with phospholipids, possible specificity to BSE.  

24. Den Hartog, Johan. Feed for food: HACCP in the animal feed industry.  Food Control. March 2003; 14(2): 95-99.  ISSN: 0956-7135
    NAL call no.:  TP372.7.F66
    Descriptors: Dutch animal feed industry, food and feed safety, GMP standard Animal Feed, BSE and dioxin, integration of HACCP, quality system, proactive approach, the Netherlands.

25. Dimcheff, Derek E.; Portis, John L.; Caughey, Byron. Prion proteins meet protein quality control.  Trends in Cell Biology. July 2003; 13(7): 337-340.  ISSN: 0962-8924
    NAL call no.:  QH573.T73
    Descriptors: protein quality control mechanisms, prion protein aggregation, pathogenesis, proteasome inhibition, neurogeneration.

26. Ducrot, Christian; Roy, Pascal; Morignat, Eric; Baron, Thierry; Calavas, Didier. How the surveillance system may bias the results of analytical epidemiological studies on BSE: Prevalence among dairy versus beef suckler cattle breeds in France. Veterinary Research Les Ulis. March-April 2003; 34(2): 185-192. ISSN:  0928-4249
    NAL call no.:  SF602.A5
    Descriptors: dairy cattle, beef cattle, BSE, disease levels, reliability of surveillance programs, data analysis, Mandatory Reporting Systems of clinically suspect bovines.

27. Ersdal, Cecilie; Simmons, Marion M.; Goodsir, Caroline; Martin, Stuart; Jeffrey, Martin. Sub-cellular pathology of scrapie: Coated pits are increased in PrP codon 136 alanine homozygous scrapie-affected sheep.  Acta Neuropathologica. July 2003; 106(1): 17-28.  ISSN: 0001-6322
    Descriptors: scrapie, sheep, sub-cellular studies, transmissible spongiform encephalopathies, heads of scrapie-affected sheep and controls were perfusion fixed with mixed aldehydes, obexes immunohistochemically labeled, PrP antibodies, electron microscopy of vagal dorsal motor nucleus, coated pits in infected animals, dystrophic neuritis, variable gliosis, plasmalemma invagination, intemalisation.

28. Ersdal, C.; Ulvund, M.J.; Benestad, S.L.; Tranulis, M.A. Accumulation of pathogenic prion protein (PrPSc) in nervous and lymphoid tissues of sheep with subclinical scrapie.  Veterinary Pathology. 2003; 40(2): 164-174. 
    NAL call no.:  41.8 P27
    Descriptors: Rygia breed sheep, pathogenic prion protein, PrPSc, immunohistochemistry, obex, cerebellum, medial retropharyngeal lymph nodes, off spring of PrPSc examined, ileal Peyer’s patch, distal jejunal lymph node, spleen, pathogenesis, prion diseases, BSE.

29. Ferguson, Neil M.; Donnelly, Christl A. Assessment of the risk posed by bovine spongiform encephalopathy in cattle in Great Britain and the impact of potential changes to current control measures.  Proceedings of the Royal Society Biological Sciences, Series B. 7 August 2003; 270(1524): 1579-1584.  ISSN: 0962-8452
    NAL call no.:  501 L84B
    Descriptors: back-calculation model to analyse data, reported clinical cases of bovine spongiform encephalopathy, BSE, analysis of demographic data, levels of human exposure estimated, discussion of possibilities of deaths from variant Cruetzfeldt-Jakob disease, vCJD. 

30. Fries, R.; Eggers, T.; Hildebrandt, G.; Rauscher, K.; Buda, S.; Budras, K.D. Autonomous nervous system with respect to dressing of cattle carcasses and its probable role in transfer of PrPres molecules.  Journal of Food Protection. 2003; 66(5), 890-895.
    NAL call no.:  44.8 J824
    Descriptors: cattle, carcasses, autonomic nervous system, cranial, cervical, and stellage ganglia, the chain of paravertebral ganglia, bovine spongiform encephalopathy, BSE, esophagus, vagus nerve, risk assessment, removal of possible infective material from the food chain.  

31. Galbraith, D.N. Transmissible spongiform encephalopathies and tissue cell culture. Cytotechnology 2003; 39(2): 117-124.  ISSN:  0920-9069
    NAL call no.:  QH585.C97
    Descriptors: TSE, prion proteins, transmission, risks of using living cells and materials for therapeutic compounds, prion biology and pathobiology, discussion of issues.

32. Geldermann, H.; Preuss, S.; Eckert, J.; Han, Y.; Ollesch, K. Analysis of polymorphic microsatellites within the bovine and ovine prion protein (PRNP) genes.  Animal Genetics. August 2003; 34(4): 283-289.  ISSN:  0268-9146
    NAL call no.:  QP98.A1A5
    Descriptors: bovine prion protein gene, microsatellite sites, sheep prion protein genes, distances between microsatellites, pylogenetic origin of alleles, BSE, scrapie, molecular genetics.

33. Gerweck, G. Ein blick auf status der tieraerzte in der lebensmittelueberwachung und tierseuchenbekaempfung. [The status of veterinarians in food surveillance and zoonosis control.]  Tieraerztliche Umschau.  January 1, 2003; 58(1): 40-43.  ISSN:  0049-3864.  In German.
    NAL call no.:  41.8 T445
    Descriptors: zoonotic disease, food safety, role of veterinarians, prion disease surveillance.

34. Ghani, Azra C.; Ferguson, Neil M.; Donnelly, Christl A.; Anderson, Roy M. Factors determining the pattern of the variant Creutzfeldt-Jakob disease (vCJD) epidemic in the UK.  Proceedings of the Royal Society Biological Sciences, Series B.  April 7, 2003; 270(1516): 689-698. ISSN:  0962-8452
    NAL call no.:  501 L84B
    Descriptors: variant of Creutzfeldt-Jakob disease, vCJD, epidemiology, human mortality prediction for 2 and 5 years, exposure to BSE, age dependent susceptibility, incubation period distribution.

35. Gonzalez, Lorenzo; Martin, Stuart; Jeffrey, Martin. Distinct profiles of PrPd immunoreactivity in the brain of scrapie- and BSE-infected sheep: Implications for differential cell targeting and PrP processing.  Journal of General Virology. May 2003; 84(5): 1339-1350.  ISSN:  0022-1317
    NAL call no.:  QR360.A1J6
    Descriptors: scrapie infected sheep, bovine spongiform encephalopathy infected sheep, PrP antibodies, on the disease causing prion protein, immunohistochemical examination of brains, 20 sheep, 4 different PrP antibodies (P4, 521.7, 505.2, R486), strain source differences, source differentiation, cell tropism, PrP processing, variations in PrPd conformation seem influenced by the cell type supporting infection, modulated by the interaction between the infectious agent and the host.

36. Gravenor, M.B.; Ryder, S.J.; Gubbins, S.; Hunter, N.; Baylis, M.; Kao, R.R. Searching for BSE in sheep: interpreting the results so far. Veterinary Record. 2003; 152(10) 298-299.
    NAL call no.:  41.8 V641
    Descriptors: sheep, screening for BSE, scrapie, confidence limit for BSE is no more that 2%, disease prevalence, UK.

37. Grigoletto, G.; Bagordo, F.; Pongolini, S.; Cantoni, A.; Cabassi, E.; Corradi, A. TSE e test diagnostici: valutazione critica e risvolti pratici nel controllo dell'encefalopatia spongiforme bovina.  [TSE and diagnostic tests: critical evaluation and practical implications for the control of bovine spongiform encephalopathy.]  Obiettivi e Documenti Veterinari. 2003; 24(3) 7-16.  In Italian.
    Descriptors: rapid diagnostic tests, BSE, Western blotting, Elisa, comparison study, use and application in Italy, existing legislation, prion diseases, cattle, disease control, Italy.

38. Gubbins, Simon; Simmons, Marion M.; Sivam, Kumar; Webb, Cerian R.; Hoinville, Linda J. Prevalence of scrapie infection in Great Britain: Interpreting the results of the 1997-1998 abattoir survey.  Proceedings of the Royal Society Biological Sciences Series B.  September 22, 2003; 270(1527): 1919-1924. ISSN:  0962-8452
    NAL call no.:  501 L84B
    Descriptors: prevalence of scrapie, sheep, human health risks, sheep transmissible spongiform encephalopathies, TSEs, slaughter plant survey, prevalence of scrapie infection, GB sheep flock of 0.22% (95% confidence interval: 0.01-0.97%).

39. Haywood, S.; Brown, D.R. Transmissible spongiform encephalopathies.  Veterinary Times. 2003; 33(2) 8-9, 10.  ISSN: 1352-9374
    Descriptors: cattle, man, sheep, TSE, CJD, scrapie, binding proteins, copper, manganese, disease transmission, environmental factors manganese. 

40. Heim, D.; Kihm, U. Risk management of transsmissible spongiform encephalopathies in Europe. Revue Scientifique et Technique Office International des Epizooties. April 2003; 22(1): 179-199. ISSN:  0253-1933
    NAL call no.:  SF 781.R4
    Descriptors: BSE, scrapie, sheep, goats, cattle, risk management decisions were inaccurate, active and passive surveillance system, ban on feeding meat-and-bone meal (MBM) to ruminants, brain and spinal column as high risk material, European measures. 

41. Hein, Wayne R.; Griebel, Philip J. A road less travelled: Large animal models in immunological research. Nature Reviews Immunology. January 2003; 3(1): 79-84. ISSN:  1474-1733
    Descriptors: many diseases, discussion of large animals as experimental models, viruses, bacteria, TSE’s.

42. Herrmann, Lynn M.; Cheevers, William P.; Davis, William C.; Knowles, Donald P.; O' Rourke, Katherine I. CD21-positive follicular dendritic cells: A possible source of PrPSc in lymph node macrophages of scrapie-infected sheep.  American Journal of Pathology. April 2003; 162(4): 1075-1081. ISSN:  0002-9440
    NAL call no.:  448.8 AM39
    Descriptors: natural sheep scrapie, lymph node analysis, presence PrPSc and macrophage or FDC markers using dual immunohistochemistry, follicular macrophages contain proteases that process full-length PrPSc to N-terminally truncated PrPSc.

43. Hetz, Claudio; Maundrell, Kinsey; Soto, Claudio. Is loss of function of the prion protein the cause of prion disorders? Trends in Molecular Medicine.  June 2003; 9(6): 237-243. ISSN:  1471-4914
    Descriptors: prion diseases, TSE, transmissible spongiform encephalopathies, mechanism of neurogeneration in spongiform encephalopathies is unknown.  

44. Hetz, C.; Soto, C. Protein misfolding and disease: The case of prion disorders.  CMLS Cellular and Molecular Life Sciences. January 2003; 60(1): 133-143.  ISSN:  1420-682X
    NAL call no:  QH301.C45
    Descriptors: TSE, review article, recent data, link between prion protein misfolding, pathogenesis of prion diseases.

45. Hlasny, J. Nektere poznatky z Britanie o historii vyzkumu deficitu horciku u prezvykavcu a BSE. [Some information from United Kingdom concerning the history about Mg- research in ruminants and BSE.] Vyzkum v Chovu Skotu. 2003; 45(1) 22-31.  ISSN:  0139-7265.  In Czech with an English summary.
    Descriptors: cattle, ruminants, BSE, prion diseases, magnesium, mineral deficiencies, United Kingdom.

46. Houston, E.F.; Gravenor, M.B. Clinical signs in sheep experimentally infected with scrapie and BSE.  Veterinary Record. 2003; 152(11): 333-334.
    NAL call no.:  41.8 V641
    Descriptors: sheep, clinical signs, subcutaneous injection, 2g SSBP/1 (scrapie group), intracerebral inoculation with 0.05 BSE brain homogenate, intravenous 0.2g BSE brain homogenate, or 550ml scrapie infected sheep blood, clinical signs compared.

47. Hunter, Nora. Scrapie and experimental BSE in sheep. British Medical Bulletin. 2003; 66: 171-183.  ISSN:  0007-1420
    Descriptors: sheep, BSE, scrapie, experimental infection, Creutzfeldt-Jacob disease, prion disease.

48. Ironside, James W. The spectrum of safety: Variant Creutzfeldt-Jakob disease in the United Kingdom.  Seminars in Hematology.  July 2003; 40(3, Suppl. 3): 16-22. ISSN:  0037-1963
    NAL call no.:  RC633.A1S44
    Descriptors: vCJD, sheep, cattle, prion diseases, BSE, scrapie, transmission, beef, animal feed, meat products, blood supply safety, bone meal products, immune response, UK.

49. Jeffrey, Martin; Martin, S.; Gonzalez, L. Cell-associated variants of disease-specific prion protein immunolabelling are found in different sources of sheep transmissible spongiform encephalopathy.  Journal of General Virology. April 2003; 84(4): 1033-1045. ISSN:  0022-1317
    NAL call no:  QR360.A1J6
    Descriptors: scrapie, BSE, prion disease, prion proteins, BSE and scrapie, intracellular accumulation patterns disease specific prions, (PrPd) lymphoreticular system (LRS)  in sheep brains clinically affected with scrapie or BSE. BSE-infected PrPARQ/ARQ sheep of different breeds compared with scrapie-infected sheep of different PrP genotypes.

50. Kaneider, Nicole C.; Kaser, Arthur; Dunzendorfer, Stefan; Tilg, Herbert; Wiedermann, Christian J.  Sphingosine kinase-dependent migration of immature dendritic cells in response to neurotoxic prion protein fragment.  Journal of Virology. May 2003; 77(9): 5535-5539. ISSN:  0022-538X
    NAL call no.:  QR360.J6
    Descriptors: TSE, circulating dendritic cells mediate neuroinvasion, prion protein expressed in myeloid dendritic cells, prion protein fragment 106-126, chemo-attractant for monocyte-derived immature dendritic cells, signaling events enzymes downstream of Gq protein, inhibition by sphingosine kinase, suggest trans-activation of sphingosine-1-phosphate-dependent cell motility by priori protein.

51. Kang, Shin Chung; Li, Ruliang; Wang, Chuanping; Pan, Tao; Liu, Tong; Rubenstein, Richard; Barnard, Geoff; Wong, Boon Seng; Sy, Man Sun. Guanidine hydrochloride extraction and detection of prion proteins in mouse and hamster prion diseases by ELISA.  Journal of Pathology. April 2003; 199(4): 534-541. ISSN:  0022-3417
    NAL call no.:  448.8 J82
    Descriptors: testing methods, invitro test for TSE, differential extraction, brain homogenates using guanidine hydrochloride followed by DELFIA (Dissociation Enhanced Lanthanide FluoroImmunoAssay), differentiate disease associated PrP isoforms without proteinase K digestion.

52. Kellar, J.A.; Lees, V.W. Risk management of the transmissible spongiform encephalopathies in North America. Revue Scientifique et Technique Office International des Epizooties. April 2003; 22(1): 201-225. ISSN:  0253-1933
    NAL call no.:  SF781.R4
    Descriptors: North American Free Trade Agreement partners, Canada, the United States of America (USA), Mexico, harmonized transmissible spongiform encephalopathy (TSE) risk management strategies, quarantine and internal surveillance, BSE, feed bans, scrapie, chronic wasting disease, transmissible mink encephalopathy, national and sub-national veterinary infrastructures, laboratory networks. 

53. Kim, Jae Il; Kuizon, Salomon; Rubenstein, Richard. Comparison of PrP transcription and translation in two murine myeloma cell lines.  Journal of Neuroimmunology. July 2003; 140(1-2): 137-142. ISSN:  0165-5728
    Descriptors: knockout mice myeloma cell lines, hybridomas, prion protein, MAbs. 

54. Kim, Nam Ho; Kim, Jae Il; Carp, Richard I.; Kim, Yong Sun. Effects of transition metals in the conversion mechanism of prion protein and in the pathogenesis of prion diseases.  Current Medicinal Chemistry, Immunology, Endocrine and Metabolic Agents. June 2003; 3(2): 149-160. ISSN:  1568-0134
    Descriptors: prion protein, cattle, CJD, scrapie, TSE, scrapie, prion diseases, pathogenesis.  

55. Kimura, Nobuhiro. BSE outbreak and feed security.  Japanese Poultry Science.  May 2003; 40(J2): J98-J104.  ISSN:  0029-0254.  In Japanese.
    NAL call no.:  47.8 N57
    Descriptors: food safety and security, public health risks, bovine spongiform encephalopathy, BSE, epidemiology, prion disease, transmission.

56. Klass, Michael R.; Hodges, Steven; Sayers, Riona; Clarke, John; Lyons, Vanessa. Testing for TSE: Mad cows, scrapie sheep and wasted deer and elk.  Abstracts of Papers American Chemical Society. 2003; 226(1-2): ANYL 10. ISSN:  0065-7727.  Note:  226th American Chemical Society National Meeting, New York, NY, USA, September 7-11, 2003
    NAL call no.:  381 AM33Pa
    Descriptors: cattle, deer, elk, sheep, scrapie, BSE, chronic wasting disease, testing, prion protein detection, ELISA, immunologic techniques, laboratory techniques, Enfer-TSE-test, Europe, Ireland, USA.

57. Kocisko, David A.; Baron, Gerald S.; Rubenstein, Richard; Chen, Jiancao; Kuizon, Salomon; Caughey, Byron. New inhibitors of scrapie-associated prion protein formation in a library of 2,000 drugs and natural products.  Journal of Virology.  October 2003; 77(19): 10288-10294. ISSN:  0022-538X
    NAL call no.:  QR360.J6
    Descriptors: prion protein, scrapie-infected mice neurobalstoma cells, scrapie strain RML, high-throughput screening assay for PrPSc, 96 well format, polyphenols (e.g., tannic acid and tea extracts), phenothiazines, antihistamines, statins, antimalarial compounds.

58. Larski, Zdzislaw. Niektore nowe dane dotyczace wirusologii i zakaznych gabczastych encefalopatii. [Some new data concerning virology and transmissible spongiform encephalopathies.]  Medycyna Weterynaryjna. 2003; 59(2): 95-99.  ISSN:  0025-8628.  In Polish.
    NAL call no.:  41.8 M463
    Descriptors: review article, lipid rafts, virons, various diseases, imbalance of trace elements and changes in antioxidant function of prion protein, BSE in sheep vs scrapie, cannibalism as a cause of BSE.

59. Lasmezas, C.I. The transmissible spongiform encephalopathies. Revue Scientifique et Technique Office International des Epizooties. April 2003; 22(1): 23-36.  ISSN:  0253-1933
    NAL call no.:  SF781.R4
    Descriptors: TSE’s, Creutzfeldt Jakob disease, CJD, etiology, prion diseases, transmission, prevention and control, scrapie,  risk management, general features of the diseases, mode of replication, pathogenesis, molecular basis of PrP accumulation.

60. Ledoux, J.M. Features of the comparative pharmacokinetics of lithium; a potential application of its use in livestock farming.  Medical Hypotheses. August 2003; 61(2): 278-281. ISSN:  0306-9877
    Descriptors: cattle, mink, lithium’s neuroprotective and neurotropic properties, treatment for sub-acute transmissible spongiform encephalopathies, proposed pharmacokinetic tests.

61. Leucht, Christoph; Simoneau, Steve; Rey, Clemence; Vana, Karen; Rieger, Roman; Lasmezas, Corinne Ida; Weiss, Stefan. The 37 kDa/67 kDa laminin receptor is required for PrPSc propagation in scrapie-infected neuronal cells. EMBO Reports. March 2003; 4(3): 290-295. ISSN:  1469-221X
    NAL call no.:  QH506.E46
    Descriptors: prions, scrapie infected, neuronal cells, infection prevention, PrPSc, laminin receptor (LRP/LR) is necessary for PrPSc propagation in vitro, LRP/LR-specific antibodies as possible therapeutic tools for transmissible spongiform encephalopathies.

62. Lewicki, Hanna; Tishon, Antoinette; Homann, Dirk; Mazarguil, Honore; Laval, Francoise; Asensio, Valerie C.; Campbell, Iain L.; DeArmond, Stephen; Coon, Bryan; Teng, Chao; Gairin, Jean Edouard; Oldstone, Michael B.A. T cells infiltrate the brain in murine and human transmissible spongiform encephalopathies.  Journal of Virology.  March 2003; 77(6): 3799-3808. ISSN:  0022-538X
    NAL call no.:  QR360.J6
    Descriptors: CD4 and CD8 T lymphocytes infiltrate parenchyma, mouse brains, intracerebral, intraperitoneal, or oral inoculation, Chandler strain of mouse scrapie, pattern compared to prion protein knockout (PrP-/-) mice, MHC class I and II molecules, elevated levels of T-cell chemokines, macrophage inflammatory protein 1beta, IFN-gamma-inducible protein 10, and RANTES, PrPSc in CNS associated with chemokines.

63. Liu, Wing Gee; Brown, Debbie A.; Fraser, Janet R. Immunohistochemical comparison of anti-prion protein (PrP) antibodies in the CNS of mice infected with scrapie.   Journal of Histochemistry and Cytochemistry.  August 2003; 51(8): 1065-1071. ISSN:  0022-1554
    NAL call no.:  381 J822
    Descriptors: transmissible spongiform encephalopathies, TSEs, tissue fixative affects, formol saline, periodate lysine paraformaldehyde,  PLP, MAbs, 6H4, 7A12 and 8H4 revealed targeted PrPsc labeling, scrapie mouse models.

64. Lloyd, T.; McCorriston, S.; Morgan, W.; Chern, W.S. (ed.); Rickertsen, K. How do markets respond to food scares? Health, Nutrition and Food Demand. 2003; 247-270.  ISBN: 0-85199-647-7
    NAL call no.:  381 J8222
    Descriptors: food scares, beef, bovine spongiform encephalopathy, impact on consumer attitudes, consumer behavior, food safety and consumer protection, economics, food consumption, food contamination, food hygiene and safety, food-intake, market economics, humans, UK.  

65. Lucassen, Ralf; Nishina, Koren; Supattapone, Surachai. In vitro amplification of protease-resistant prion protein requires free sulfhydryl groups.  Biochemistry April 15 2003; 42(14): 4127-4135.  ISSN:  0006-2960
    NAL call no.:  381 B523
    Descriptors: PrPSc, prion misfolding, molecular mechanism of misfolding, in-vitro PrPSc amplification techniques, scrapie infected brain homogenate, in vitro amplification with Syrian hamster Sc237PrPSc, pH7 and CD-1 mouse RML PrPSc pH6, thiolate-specific alkylating agent N-ethylmaleimide (NEM), reversible thiol-specific blockers p-hydroxymercuribenzoic acid (PHMB) and mersalyl acid inhibited PrPSc amplification.

66. Mabbott, Neil A.; Young, Janice; McConnell, Irene; Bruce, Moira E. Follicular dendritic cell dedifferentiation by treatment with an inhibitor of the lymphotoxin pathway dramatically reduces scrapie susceptibility.  Journal of Virology. June 2003 2003; 77(12): 6845-6854. ISSN:  0022-538X
    NAL call no.:  QR360.J6
    Descriptors: transmissible spongiform encephalopathies, TSEs, mouse scrapie animal model of disease, dendritic cells, prion protein for replication in lymphoid tissue and subsequest neuroinvasion, lymphotoxin beta receptor-immunoglobulin fusion protein (LTbetaR-Ig), temporary dedifferentiation, intraperitoneal scrapie inoculation blocked early accumulation of PrPSc in spleen, reduced disease susceptibility, 28 and 49 days, routes of exposure, possible early intervention.

67. Mainsant, P. Meat consumption before and after BSE in France and the European Union.  Sciences des Aliments. 2003; 23(1): 37-39.  ISSN:  0240-8813
    NAL call no.:  TX341.S34
    Descriptors:  consumer responses, meat and meat product consumption, bovine spongiform encephalopathies, beef cattle, comparison study, EU.

68. Manuelidis, Laura. Transmissible encephalopathies: Speculations and realities.  Viral Immunology. Summer 2003; 16(2): 123-139. ISSN:  0882-8245
    Descriptors: scrapie, CJD, BSE, prion theory, review article, PrP, transmission factors, pathology, response to infectious agent, possible viral caused disease.

69. Matthews, D.; Cooke, B.C. The potential for transmissible spongiform encephalopathies in non-ruminant livestock and fish.  Revue Scientifique et Technique Office International des Epizooties. April 2003; 22(1): 283-296. ISSN: 0253-1933
    NAL call no.:  SF781.R4
    Descriptors: pigs, poultry, susceptibility to BSE agent, parenteral challenge causes infection in pigs, oral exposure to BSE-infected cattle brain, oral challenge with sheep scrapie, chickens show resistant to oral challenge with sheep scrapie.

70. Matthews, D. BSE: A global update.  Society for Applied Microbiology Symposium Series. 2003; (32): 120S-125S. ISSN:  1467-4734
    NAL call no.:  QR1.S64
    Descriptors: BSE, active surveillance in Europe, Britain’s epidemic, British feed controls, epidemiology, cattle, European Commission's Scientific Steering Committee considering risks of importing BSE, consumer concerns, dispersal of infectivity from European countries was widespread.

71. McCrea, D. Risk communication of the transmissible spongiform encephalopathies.  Revue Scientifique et Technique Office International des Epizooties. April 2003; 22(1): 251-257. ISSN:  0253-1933
    NAL call no.:  SF781.R4
    Descriptors: risk communication, public, prion disease, epidemiology, disease prevention and control, transmission, transmissible spongiform encephalopathy, food products, food safety, risk communication, definitions, goals.

72. McKintosh, Edward; Tabrizi, Sarah J.; Collinge, John. Prion diseases.  Journal of Neurovirology. April 2003; 9(2): 183-193. ISSN:  1355-0284
    Descriptors: BSE, vCJD, history, epidemiology, prion diseases, TSE, current research human prion disorders.

73. Miele, G.; Blanco, A.R. Alejo; Baybutt, H.; Horvat, S.; Manson, J.; Clinton, M. Embryonic activation and developmental expression of the murine prion protein gene. Gene Expression. 2003; 11(1): 1-12. ISSN:  1052-2166
    NAL call no.:  QH450.G46S
    Descriptors: prion protein, PrP mRNA expression, murine embryos and various adult tissues, expression PrP RNA not in adult kidney and liver, investigated effected superoxide radicalin cultured neuroblastoma and astrocyte cells, suggest that PrPC is part of cellular antioxidant defense mechanism.

74. Morley, R.S.; Chen, S.; Rheault, N. Assessment of the risk factors related to bovine spongiform encephalopathy.  Revue Scientifique et Technique Office International des Epizooties. April 2003; 22(1): 157-178.  ISSN:  0253-1933
    NAL call no.:  SF781.R4
    Descriptors: status of BSE, cattle populations, risk assessment, surveillance criteria, International Animal Health Code, consumption of meat-and–bone-meal (MBM) by cattle, importation of cattle, possible contamination of MBM, livestock population structure, rendering processes, animal feeding practices. Application of the OIE, BSE guidelines, costs and losses with introduction and establishment of BSE in other countries, Canada.   

75. Nishida, Yuzo. Elucidation of endemic neurodegenerative diseases: A commentary. Zeitschrift fuer Naturforschung Section C, Journal of Biosciences. September-October 2003; 58 (9-10): 752-758. ISSN: 0939-5075
    NAL call no.:  QH301.Z4
    Descriptors: scrapie, CJD, chronic wasting disease, soil metal ion levels, copper, manganese, iron, aluminum, cellular accumulation of metallic ions, acid rain effects on solubility, iron-overload syndrome, hydrogen peroxide, prion isoforms, PrPc and PrPSc, Iceland, Slovakia, Colorado.

76. Nishida, Noriyuki; Sakaguchi, Suehiro; Katamine, Shigeru. Prion disease and antiprion substance.  Journal of Pharmacological Sciences. 2003; 91(Supplement I): 48P. ISSN: 1347-8613.  Note:  76th Annual Meeting of the Japanese Pharmacological Society, Fukuoka, Japan, March 24-26, 2003
    Descriptors: anti-prion substance, Congo Red, prion disease, drug screening system, cell culture model, histology and cytology techniques.

77. Nunziante, Max; Gilch, Sabine; Schaetzl, Hermann M. Essential role of the prion protein N terminus in subcellular trafficking and half-life of cellular prion protein.  Journal of Biological Chemistry. February 7, 2003; 278(6): 3726-3734.  ISSN:  0021-9258
    NAL call no.:  381 J824
    Descriptors: prion protein life history, biochemistry, glycosylphosphatidylinositol anchor, prion-protein N-terminus, cellular half-life, transmissible spongiform encephalopathies.

78. O'Rourke, Katherine I.; Knowles, Donald P.; Baszler, Timothy V.; Parish, Steven M.  Methods for detection of prion protein as an indication of transmissible spongiform encephalophathies. Official Gazette of the United States Patent and Trademark Office Patents. [e-file] Feb. 4, 2003; 1267(1): No Pagination.  ISSN:  0098-1133
    NAL call no.:   T223.A21
    Descriptors: PrPSc, prion protein, detection methods, diagnostic techniques. infected live animals, postmortem detection methods, third eyelid-associated lymphoid tissue, monoclonal antibodies, conserved epitope of PrPSc protein, fixed or frozen tissue.

79. Ochel, H. J.; Gademann, G.; Trepel, J.; Neckers, L. Modulation of prion protein structural integrity by geldanamycin.  Glycobiology.  September 2003; 13(9): 655-660.  ISSN:  0959-6658
    NAL call no.:   QP552.G59G593
    Descriptors: prion protein, transmissible spongiform encephalopathies, HSP90 inhibitors, geldanamycin or radicicol effects, eukaryotic cells, tunicamycin, bands of western blot analysis.

80. Office International des Epizooties. Risk analysis of prion diseases in animals. Revue Scientifique et Technique Office International des Epizooties. 2003; 22(1), 344 pp.  ISSN:  0253-1933.  In English, French, and Spanish.
    NAL call no.:   SF781.R4
    Descriptors: BSE, TSE, scrapie, prion diseases, current and established knowledge, risk assessment, management in different countries, diagnosis and disease prevention.    

81. Ozawa, T.; Lopez-Villalobos, N.; Blair, H.T. Beef traceability systems in Japan: How should New Zealand prepare? Proceedings of the New Zealand Society of Animal Production. 2003; 63: 49-52.  ISSN:  0370-2731
    NAL call no.:   49.9 N483
    Descriptors: BSE, tracing systems, farm to consumer systems, not affective in Japan or other countries, question of expenses vs expected financial to NZ beef producers, Ministry of Agriculture, Forestry and Fisheries, National Federation of Agricultural Cooperative Association, New Zealand Meat Board.  

82. Ozawa,Y. Risk management of transmissible spongiform encephalopathies in Asia. Revue Scientifique et Technique Office International des Epizooties. April 2003; 22(1): 237-249. ISSN: 0253-1933
    NAL call no.:   SF781.R4
    Descriptors: questionnaire-based survey, distributed to the Office International des Epizooties Member Countries in Asia, risk management for transmissible spongiform encephalopathies, 16 responses, risk analysis not in 10 countries, ruminant origin feed stuff imported into Asia, upgrading of surveillance and notification programs recommended, BSE, scrapie.

83. Peelman, L.J.; van Poucke, M. Een eerste bepaling van de PRNP-genotypenfrequenties bij de voornaamste schapenrassen in Belgie. [The first determination of PRNP genotype frequency in the most important sheep breeds in Belgium.] Vlaams Diergeneeskundig Tijdschrift. 2003; 72(1): 20-26. In Dutch with an English summary.
    NAL call no.:   41.8 V84
    Descriptors: scrapie, sheep, selection of scrapie resistant sheep, ARR allele, sampled most breeds use in Belgium, 854 animals, VrQ/VrQ and ARR/ARR genotype.

84. Pennington, H. Science, governments and microbes: Food safety in the 21st century.  FEMS Congress of European Microbiologists Abstract Book. 2003; (1): 1. Note:  1st Federation of European Microbiological Societies (FEMS) Congress of European Microbiologists, Ljubljana, Slovenia, June 29-July 03, 2003.
    Descriptors: research, governmental policies, infectious organisms, food borne pathogens, bacteria, E. coli O157, production animals, prions, viruses, BSE.

85. Periago, P.M.; Fernandez, A.; Collado, J.; Martinez, A. Note: Use of a distribution of frequencies model to interpret the tailed heat inactivation curves of prions. Food Science and Technology International. February 2003; 9(1): 29-32. ISSN:  1082-0132
    NAL call no.:   TP368.F66S
    Descriptors: BSE, bovine spongiform encephalopathy, prion-disease, Weibull models, two parameter empirical model, mathematical and computer techniques for statistical analysis, canned food industry, kinetics, prion inactivation curves, different temperatures under different conditions, Europe.

86. Polak, M.P.; Zmudzinski, J.F.; Larska, M.; Rozek,W.; Kozaczynski, W.; Reichert, M.  Skutecznosc systemu monitorowania BSE w Polsce na przykladzie pierwszych wykrytych przypadkow choroby. [Efficacy of the BSE monitoring system in Poland [analysis of the first four cases].  Zycie Weterynaryjne. 2003; 78: 2, 97-99.  ISSN:  0137-6810.  In Polish with an English summary.
    NAL call no.:   SF604.Z9
    Descriptors: cattle, BSE, bovine spongiform encephalopathy, case reports, disease monitoring, immunological techniques, public health concerns, Poland.  

87. Prendergast, D.M.; Sheridan, J. J.; Daly, D. J.; McDowell, D.A.; Blair, I.S. Dissemination of central nervous system tissue from the brain and spinal cord of cattle after captive bolt stunning and carcass splitting.  Meat Science. December 2003; 65(4): 1201-1209. ISSN:  0309-1740
    NAL call no.:   TX373.M4
    Descriptors: BSE, cattle, measures to exclude specified risk materials from human food chain, CNS tissue dissemination, slaughter practices, captive bolt stunning, carcass splitting, CNS material contamination of meat.

88. Polak, Miroslaw P.; Larska, Magdalena; Zmudzinski, Jan F. Nowe szybkie testy do diagnostyki post mortem BSE. [New rapid tests for BSE post mortem diagnosis.]  Medycyna Weterynaryjna. 2003; 59(10): 876-878.  ISSN:  0025-8628. In Polish.
    NAL call no.:   41.8 M463
    Descriptors: cattle, BSE, rapid diagnostic test comparison, screening methods and techniques, sensitivity, specificity, limiting detectable levels, PrPSc, prions, Scientific Steering Committee of the European Commission, post-mortem testing.

89. Purdey, Mark. Does an infrasonic acoustic shock wave resonance of the manganese 3+ loaded/copper depleted prion protein initiate the pathogenesis of TSE?  Medical Hypotheses.  June 2003; 60(6): 797-820.  ISSN:  0306-9877
    Descriptors: copper, Cu, Mn3+, hyper-polarization, paramagnetic-status, piezoelectric atomic structure, cupro-protein expression, transmissible spongiform encephalopathy, prion diseases, UV radiation, acoustic radiation, circadian auditory pathway, electromagnetic superexchange, electron phonon coupling, endogenous electromagnetic energy, geomagnetic radiation, infrasound rich environment, tetonic disturbances, supersonic airplanes, ratios of high MN/low copper and low zinc of mammals brains, 2 stage TSE pathogenesis initiated when MN substitutes for vacant Cu domain on PrPC (sleeping) when infrasonic shock the atomic structure of Mn3+ starts the distortion of the protein, Cu prions replaced by hyperpolarized Mn 3+ prions, self perpetuating 'cluster bombs' of free radical mediated neurodegeneration.  

90. Rachidi, Walid; Mange, Alain; Senator, Abderrahmene; Guiraud, Pascale; Riondel, Jacqueline; Benboubetra, Mustapha; Favier, Alain; Lehmann, Sylvain. Prion infection impairs copper binding of cultured cells.  Journal of Biological Chemistry. April 25 2003; 278(17): 14595-14598. ISSN:  0021-9258
    NAL call no.:   381.J824
    Descriptors: TSE, molecular mechanism of neurogeneration, 64CU, radioactive copper, prion infected cells, reduction in copper binding, prion infection modulates copper content at a cellular level, modification of copper homeostasis has determinant role in neuropathology.

91. Richard, Marlene; Biacabe, Anne Gaelle; Streichenberger, Nathalie; Ironside, James West; Mohr, Michel; Kopp, Nicolas; Perret, Liaudet Armand. Immunohistochemical localization of 14.3.3 zeta protein in amyloid plaques in human spongiform encephalopathies.  Acta Neuropathologica. March 2003; 105(3): 296-302.  ISSN: 0001-6322
    Descriptors: MV2 CJD, kuru, VV2cjd, vCJD, Gerstmann-Straussler-Scheinker, Alzheimer, paraffin-embedded brain section, different subtypes of brain amyloid plaques, comparison of PrPSc and 14.3.3 zeta deposits presence, data suggests, 14.3.3 zeta protein could interact with PrP, other components of PrPSc deposites in CJD, immunostaining.

92. Rico, Andre. Prion: Toxic or infectious agent?  Medical Hypotheses.  February 2003; 60(2): 209-214.  ISSN:  0306-9877
    Descriptors: TSE, caused by deficiency of chemo-defense system (CDS), unable to destroy or eliminate PrPsc, immune defense system (IDS) accommodates PrPsc as an inert particle that is moved through lymphoreticular system, PrPSc acts as cellular toxic disruptor of post-translational phase of PrP biosynthesis. 

93. Ridley, Rosalind M. What would T. H. Huxley have made of prion diseases? Molecular Biotechnology. July 2003; 24(3): 243-256. ISSN:  1073-6085
    NAL call no.:   TP248.13.M65
    Descriptors: speculating on T.H. Huxle’s thoughts regarding transmissible spongiform encephalopathies, scientific debate over prion hypotheses.  

94. Roels, Stefan L.M. F.; De Meyer, Gaelle; Tedik, Kamile; Foubert, Raphael; Vanopdenbosch, Emmanuel. Variation of mass (volume) taken with the calibrated syringe and of the results provided by the Bio-Rad PlateliaTM BSE test upon storage of brainstem samples at -20degrees C.  Animal Research. November-December 2002(2003); 51(6): 493-499. ISSN: 1627-3583
    Descriptors: suspected BSE brainstem material, sampling with a kit calibrated syringe, quick PlateliaTM test from Bio-Rad, decrease in optical density after 1 week cold storage, density change did not affect diagnosis of BSE. 

95. Rudyk, Helene; Knaggs, Michael H.; Vasiljevic, Snezana; Hope, James; Birkett, Chris; Gilbert, Ian H. Synthesis and evaluation of analogues of congo red as potential compounds against transmissible spongiform encephalopathies. European Journal of Medicinal Chemistry.  June 2003; 38(6): 567-579.  ISSN:  0223-5234
    Descriptors: anti-TSE compounds, analogues, Congo red 4, 6, 8, diazonium salts,  symmetrical bis azoic dyes 14-19, 21-22, 24 and 26-29 as their sodium salts, molecular modeling, potential structure activity relationships.

96. Salman, Mo D. Chronic wasting disease in deer and elk: Scientific facts and findings.  Journal of Veterinary Medical Science. July 2003; 65(7): 761-768. ISSN:  0916-7250
    NAL call no.:   SF604.J342
    Descriptors: deer, elk, chronic wasting disease, CWD, disease history, pathogenesis, susceptibility of animals, transmission, potential origins, diagnostic methods, control strategies, economic impact, food and food safety, public health risks, infectivity to humans, North America.

97. Schreuder, B.E.C.; Somerville, R.A. Bovine spongiform encephalopathy in sheep?   Revue Scientifique et Technique Office International des Epizooties. April 2003; 22(1): 103-120.  ISSN:  0253-1933 ISSN:  0253-1933
    NAL call no.:   SF781.R4
    Descriptors: BSE infected sheep hypothetical, risk management and pre-emptive measures, experimental BSE in sheep, differentiating BSE and scrapie in same host, classical strain typing in a mouse model.   

98. Sellier, P. Protein nutrition for ruminants in European countries, in the light of animal feeding regulations linked to bovine spongiform encephalopathy.  Revue Scientifique et Technique Office International des Epizooties. April 2003; 22(1): 259-269.  ISSN:  0253-1933
    NAL call no.:   SF781.R4
    Descriptors: BSE, protein in production animal diets, new sources of protein, minerals and lipids in animal diets, manufactured concentrates, vegetals as feed source alternatives, feed regulations, implementation and management, Europe, World.   

99. Simoneau, Steve; Haik, Stephane; Leucht, Christoph; Dormont, Dominique; Deslys, Jean Philippe; Weiss, Stefan; Lasmezas, Corinne. Different isoforms of the non-integrin laminin receptor are present in mouse brain and bind PrP.  Biological Chemistry.  February 2003; 384(2): 243-246. ISSN:  1431-6730
    NAL call no.:   QP501.B56
    Descriptors: prion protein, mouse brain fractions, laminin receptor, isoforms interaction with prion protein, physiological role, laminin receptor/PrP interaction in the brain, relevance for transmissible spongiform encephalopathies.

100. Smith, Peter G.; Bradley, Ray. Bovine spongiform encephalopathy (BSE) and its epidemiology. British Medical Bulletin. 2003; 66: 185-198.  ISSN:  0007-1420
     Descriptors: BSE, cattle, food safety, sheep, humans, processed animal waste, prion diseases, epidemiology, UK.

101. Smith, Peter G. The epidemics of bovine spongiform encephalopathy and variant Creutzfeldt-Jakob disease: Current status and future prospects.  Bulletin of the World Health Organization.  2003; 81(2): 123-130.  ISSN:  0042-9686
     NAL call no.:   449.9 W892B
     Descriptors: BSE, epidemic, Europe, UK, control measures, EU, Switzerland, testing post mortem, tests for live animals needed, risks small and diminishing in Europe, 150 diagnosed with vCJD, control of iatrogenic transmission via blood transfusion or contaminated surgical instruments, new tests, decontamination methods needed. 

102. Speare, Jonathan O.; Rush, Thomas S. III; Bloom, Marshall E.; Caughey, Byron. The role of helix 1 aspartates and salt bridges in the stability and conversion of prion protein.  Journal of Biological Chemistry. April 4 2003; 278(14): 12522-12529. ISSN:  0021-9258
     NAL call no.:   381.J824
     Descriptors: pathogenesis,  transmissible spongiform encephalopathies, conversion of PrPsen to PrPres, constructed mutants of hamster prion protein, replacing aspartic acids, salt bridges, cell-free conversion data, Asp-144 and Asp-147 and the respective salt bridges stabilize PrPsen from converting to PrPres. 

103. Stoltze, L.; Rezaei, H.; Jung, G.; Grosclaude, J.; Debey, P.; Schild, H.; Rammensee, H.G. CD4+ T cell-mediated immunity against prion proteins.  CMLS—Cellular and Molecular Life Sciences. March 2003; 60(3): 629-638.  ISSN:  1420-682X
     NAL call no.:   QH301.C45
     Descriptors: prion protein, amino sequences of PrP, differences in amino acid sequence, presentation of distinct peptides on major histo-compatibility complex class II molecules, activation of specific CD4+ T cells, effective immune response against foreign PrP, antibody production, distinguish self and foreign. 

104. Tahiri, Alaoui Abdessamad; Bouchard, Mario; Zurdo, Jesus; James, William.  Competing intrachain interactions regulate the formation of beta-sheet fibrils in bovine PrP peptides.  Protein Science. March 2003; 12(3): 600-608. ISSN: 0961-8368
     NAL call no.:   QD431.A1P78
     Descriptors: bovine prion protein biochemistry, pathogenesis of TSE, PrPC helix converted to aberrant beta-sheet-dominated form (PrPSc), peptide analysis.  Native PrPC helix 1 might inhibit the strong intrinsic beta-sheet-forming propensity of sequences immediately N-terminal to the globular core of PrPC, by keeping in place intrachain interactions that would prevent these amyloidogenic regions from triggering aggregation.

105. Taylor, D. M.; Woodgate, S.L. Rendering practices and inactivation of transmissible spongiform encephalopathy agents. Revue Scientifique et Technique Office International des Epizooties. April 2003; 22(1): 297-310. ISSN: 0253-1933
     NAL call no.:   SF781.R4
     Descriptors: BSE, scrapie, meat and bone meal, rendering of infected animal tissues, safety of tallow and tallow by-products, relationship between BSE and variant Creutzfeldt-Jakob disease, vCJD, UK.

106. Terry, L.A.; Marsh, S.; Ryder, S.J.; Hawkins, S.A.C.; Wells, G.A.H.; Spencer, Y.I.  Detection of disease-specific PrP in the distal ileum of cattle exposed orally to the agent of bovine spongiform encephalopathy. Veterinary Record. 2003; 152(13): 387-392.
     NAL call no.:   41.8 V641    
     Descriptors: BSE, cattle, distal ileum, PrPSc, mesemteric lymph nodes, experimental orally dosed animals, naturally occurring clinical cases, Peyer’s patches, differences, immunohistochemistry, macrophages. 

107. Thackray, Alana M.; Madec, Jean Yves; Wong, Edmond; Morgan, Warren Robert; Brown, David R.; Baron, Thierry; Bujdoso, Raymond. Detection of bovine spongiform encephalopathy, ovine scrapie prion-related protein (PrPSc) and normal PrPc by monoclonal antibodies raised to copper-refolded prion protein.  Biochemical Journal.  15 February 2003; 370(1): 81-90.  ISSN:  0264-6021
     NAL call no.:   QP 501.B64
     Descriptors: BSE, scrapie, prion proteins, monoclonal antibodies for copper-refolded PrP, diagnosis of normal and abnormal forms of protein, disease-susceptible allelic form V136R154Q171 ('VRQ'; where single-letter amino-acid notation has been used) and disease-resistant allelic form A136R154R171 ('ARR') of recombinant ovine PrPc, reaction unglycosylated and monoglycosylated forms of PrPSc from prion-infected tissue samples, different species tested by Western blot, distinquished  between bovine spongiform encephalopathy & scrapie PrPSc from experimentally infected sheep due to different electrophoretic mobilities.

108. Thomzig, Achim; Kratzel, Christine; Lenz, Gudrun; Krueger, Dominique; Beekes, Michael. Widespread PrPSc accumulation in muscles of hamsters orally infected with scrapie. EMBO Reports. May 2003; 4 (5): 530-533.  ISSN:  1469-221X
     NAL call no.:   QH506.E46
     Descriptors: hamsters, animal model of disease, scrapie, BSE, chronic wasting disease, TSE, spongiform encephalopathies, prion proteins, PrPSc, western blotting, skeletal muscle levels, forelimb, hindlimb, head, back, shoulder, tongue, peripheral routing of infection, question whether muscles contain the infectious agent.

109. Travis, Dominic; Miller, Michele. A short review of transmissible spongiform encephalopathies, and guidelines for managing risks associated with chronic wasting disease in captive cervids in zoos.  Journal of Zoo and Wildlife Medicine. June 2003; 34 (2): 125-133. ISSN:  1042-7260
     NAL call no.:   SF601.J6
     Descriptors: prion diseases, transmissible spongiform encephalopathies, TSE, scrapie, kuru, variant Creutzfeld-Jakob disease, review article, zoo and wildlife veterinarians, wildlife biologists, chronic wasting disease, cervids, risk management strategies, disease prevention.

110. Trevitt, Clare R.; Singh, Pramil N. Variant Creutzfeldt-Jakob disease: Pathology, epidemiology, and public health implications.  American Journal of Clinical Nutrition. September 2003; 78(3 Supplement): 651S-656S. ISSN:  0002-9165
     NAL call no.:   389.8 J824
     Descriptors: BSE, vCJD, transmissible spongiform encephalopathies, cattle, sheep, scrapie, prion diseases, review, outline public health implications, data, emphasizing preventative measures, areas of research for screening and diagnosis.

111. Van Gelderen, C.; Gimeno, E.J.; Schudel, A.A. Bovine spongiform encegphalopathy in South America: A regional preventive approach.  Revue Scientifique et Technique Office International des Epizooties. April 2003; 22(1): 227-236.  ISSN:  0253-1933
     NAL call no.:   SF781.R4
     Descriptors: bovine spongiform encephalopathy, BSE, cattle, prion diseases, disease prevention, safeguarding animal health, preventative action adopted in 1989, region is free of BSE, South American programs, harmonizing BSE prevention programs, maintenance of disease free status.

112. Watt, Nicole T.; Hooper, Nigel M. The prion protein and neuronal zinc homeostasis.  Trends in Biochemical Sciences.  August 2003; 28(8): 406-410.  ISSN:  0968-0004
     NAL call no.:   QH345.T73
     Descriptors: prion protein, PrP, transmissible spongiform encephalopathies, TSE, binding of metal ions, protein role may be in zinc concentration management, effects of conformation change.

113. Weiss, D. Beef is back? Rindfleischmarkt auf niedrigerem Niveau stabilisiert.  [Beef is back? Stabilization of the beef market at a lower level.]  May. 2003; 31(1): 25-27.  ISSN:  0341-5155.  In German.
     Descriptors: cattle, beef bulls, level of veal and beef consumption, BSE crisis, producer prices, European Countries, Germany.

114. Wells, Gerald A.H.; Hawkins, Stephen A. C.; Austin, Anthony R.; Ryder, Stephen J.; Done, Stanley H.; Green, Robert B.; Dexter, Ian; Dawson, Midchael; Kimberlin, Richard H. Studies of the transmissibility of the agent of bovine spongiform encephalopathy to pigs. Journal of General Virology. April 2003; 84(4): 1021-1031. ISSN:  0022-1317
     NAL call no.:   QR360.A1J6
     Descriptors: BSE, transmissibility to pigs, parenteral inoculation via intracranial, intravenous, and intraperitoneal routes, incubation period of 69-150 weeks, infectivity testing, bioassay in inbred mice, infectivity found in pig’s stomach, jejunum, distal ileum, pancreas, CNS, orally exposed animals not infected. 

115. Wichert von Holten, S. Psychosoziale Belastungen der Menschen bei Massentoetungen von Nutztieren.  [Psycho-social stress in humans at mass slaughter of farm animals.] DTW Deutsche Tieraerztliche Wochenschrift.  May 2003; 110(5): 196-199. ISSN:  0341-6593.  In German.
     NAL call no.:   41.8 D482
     Descriptors: veterinarians, concerned people, slaughter teams, mass slaughter, contaminated animals, BSE, MKS epidemics, emotional consequences, pastoral care task force service, crisis and stress, Lower Saxony.  

116. Williams, E.S.; Miller, M. W. Transmissible spongiform [encepiharopathies]  (encephalopathies) in non-domestic animals: Origin, transmission and risk factors. Revue Scientifique et Technique Office International des Epizooties. April 2003; 22(1): 145-156.  ISSN:  0253-1933
     NAL call no.:   SF781.R4
     Descriptors: transmissible spongiform encephalopathies, TSE, scrapie, bovine spongiform encephalopathy, BSE, transmissible mink encephalopathy, chronic wasting disease, deer, elk, oral transmission, impact on free ranging cervids, captive animal feed stuffs as sources, recommended surveillance of domestic and wild, infectious agent.

117. Wolferstan, Frances. Slow neurodegeneration and transmissible spongiform encephalopathies/prion diseases Hypothesis: A cycle involving repeated tyrosine kinase A activation could drive the development of TSEs. Medical Hypotheses.  January 2003; 60(1): 52-64. ISSN: 0306-9877
     Descriptors: TSE theory, prion proteins, nerve regeneration and repair, prions modulate tryosine kinase activation, abnormal prion isoforms, damaged and release fragments of prion PrP106-126, stimulate release of nerve growth factor, which activates tyrosine kinase once more, setting up the vicious spiral of slow neurodegeneration found in transmissible spongiform encephalopathies.

118. Ye, X; Carp, R.I.; Meeker, H.C.; Scallet, A.C. Identification and detection of transmissible spongiform encephalopathies.  Current Medicinal Chemistry, Immunology, Endocrine, and Metabolic Agents.  June 2003; 3(2): 95-111.  ISSN:  1568-0134
     Descriptors: TSE, sheep, goats, scrapie, prion disease, ELISA, Western blot, brain biopsy.

2002

1. Abiola, Oduola O.; Iyegbe, Conrad; Lantos, Peter; Plomin, Robert; Anderton, Brian H.; Whatley, Stephen A. Profound sex-specific effects on incubation times for transmission of bovine spongiform encephalopathy to mice. Intervirology. 2002; 45(1): 56-8 ISSN: 0300-5526.
   Abstract: Four strains of mice were inoculated intracerebrally with a primary isolate of bovine spongiform encephalopathy (BSE) and the cloned mouse-adapted scrapie strain ME7. Clinical prion disease diagnosis was made at the appearance of three or more neurological symptoms and their persistence for 3 consecutive weeks and confirmed by neuropathological criteria. For BSE, incubation periods were profoundly different between the sexes in all four mouse strains, being longer in the females. In contrast, ME7 scrapie incubation times were similar between the sexes. Our results indicate that sex-specific processes are involved in the course of primary BSE transmission. Research into this phenomenon may provide clues to the prophylaxis of BSE and have possible implications for new variant Creutzfeldt-Jakob disease in humans.
   NAL call no. QR355.I5
   Descriptors: BSE, scrapie strain ME7, 4 strains of mouse models, inbred C57BL, inbred DBA; experimental infections, brain inoculation, sex differences, NvCreutzfeldt-Jakob Disease, prion physiology.

2. Agerholm, J. S.; Tegtmeier, C. L.; Nielsen, T. K. Survey of laboratory findings in suspected cases of bovine spongiform encephalopathy in Denmark from 1990 to 2000. APMIS, Acta Pathologica, Microbiologica, et Immunologica Scandinavica 2002 Jan; 110(1): 54-60 ISSN: 0903-4641.
   Abstract: A survey of the laboratory findings in suspected cases of bovine spongiform encephalopathy (BSE) in Denmark from 1 June 1990 to 31 December 2000 is presented. During this period BSE was a notifiable disease, and the heads of suspected cases were submitted according to the legislation on BSE. A total of 176 submissions were made, mostly from bovines with neurological disorders and mainly during the last 3 years of this period. Lesions or other laboratory findings consistent with severe neurological disorders were found in 115 cases. The most frequent diagnosis was encephalic listeriosis (35.8% of submissions) followed by other forms of inflammatory lesions. A wide range of lesions were diagnosed less prevalent. BSE was diagnosed twice. The first case occurred in an imported cow in 1992, while the second confirmed case was diagnosed in a native cow in February 2000. A marked increase in the number of submissions occurred following the detection of BSE in February 2000.
   NAL call no. QR1.A6
   Descriptors: cattle, BSE, checking bovines exhibiting neurological disorders, Denmark.

3. Aguzzi, Adriano. Die Prion-Hypothese und Prionen-Krankheiten des Menschen. [The prion hypothesis and the human prion diseases]. Berliner und Munchener Tierarztliche Wochenschrift 2002 Mar-Apr; 115(3-4): 91-8 ISSN: 0005-9366. In German.
   Abstract: Our understanding of the pathogenesis of the transmissible spongiform encephalopathies (TSE) has made terrific headway over the past 40 years and some scientists are even of the opinion that this group of diseases belongs to the neurodegenerative syndromes best understood. On the other hand, the investigation of TSE has led to a multitude of unexpected and surprising results and consequently has initiated impassioned discussions among scientists. Although the human forms of TSE are very rare, the wildfire-like spread of the bovine spongiform encephalopathy (BSE) raises the pressing question as to whether BSE is communicable to humans. This overview summarizes some current hypotheses about the nature of the infectious agent and about the pathogenesis of the damage of the central nervous system.
   NAL call no. 41.8 B45
   Descriptors: pathogenesis, transmissible spongiform encephalopathies, BSE, infectous agent, prion diseases, cattle, humans.

4. Alperovitch, Annick; Will, Robert G. Predicting the size of the vCJD epidemic in France. Comptes Rendus Biologies. Janvier, 2002; 325 (1): 33-36.
   DOI: 10.1016/S1631-0691(02)01410-5
   NAL call no. Q2 C6
   Descriptors: NvCJD, Creutzfeldt-Jakob disease, BSE, France, France/UK ratios of disease, exposure to high risk foods, epidemiology.

5. Anand, P. Public health. Decision-making when science is ambiguous. Science. 2002 Mar 8; 295(5561): 1839. ISSN: 1095-9203.
   NAL call no. 470 SCI2
   Descriptors: BSE, bovine spongiform encephalopathy, prevention and control, public health, disease transmission, policy making, probability.

6. Anonymous. Embryo transfer and BSE. Veterinary Record. London : The British Veterinary Association. Mar 23, 2002. v. 150 (12) p. 357. ISSN: 0042-4900.
   NAL call no. 41.8 V641
   Descriptors: cattle, embryo transfer, BSE, bovine spongiform encephalopathy, disease transmission, UK.

7. Anonymous. Harvard study finds BSE poses little threat to U.S. consumers, agriculture. Journal of the American Veterinary Medical Association. 2002 Feb 1; 220(3): 279-80. ISSN: 0003-1488.
   NAL call no. 41.8 AM3
   Descriptors: animal feed standards, BSE, bovine spongiform encephalopathy, prevention and control, US laws.

8. Aucouturier, Pierre. Dendritic cells and prion propagation. The Scientific World Journal [online] Feb, 2002; 1 (Cited April 4, 2002): 38-40.
   Descriptors: prion protein, movement of protein from the intestinal system, dendretic cells, lymph system.

9. Aupperle, H.; Lucker, E.; Overhoff, M.; Schoon, H. A. Verfahren zum Nachweis von im Hinblick auf die BSE unerwunschten Zutaten in Fleischerzeugnissen: 6. Immunhistologischer Nachweis von zentralem und peripherem Nervengewebe in Fleischerzeugnissen. [Procedures for the unwanted ingredients in meat products with regard to BSE: immunohistochemical procedures for the detection of central and periphere nervous tissue in meat products.] Fleischwirtschaft. 2002., 82: 3, 100-104; 27 Ref. In German with an English summary.
   NAL call no. 280.38 F62
   Descriptors: BSE, bovine spongiform encephalopathy, diagnostic techniques, CNS tissue in muscle tissue, food safety, prevention, quality control.

10. Balen, Adam. Is there a risk of prion disease after the administration of urinary-derived gonadotrophins? Human Reproduction. Oxford. July, 2002; 17 (7): 1676-1680.
    DOI: 10.1093/humrep/17.7.1676-a/
    NAL call no. QP251 H85
    Descriptors: prion protein, scrapie, bovine spongiform encephalopathy, BSE, Creutzfeldt Jakob disease, CJD, contaminated urine in pharmaceuticals, bovine serum.

11. Balter, Michael BSE in sheep? Humiliated lab fights to save face. Science. 2002 Feb 1; 295(5556): 792-3 ISSN: 1095-9203
    NAL call no. 470 SCI2
    Descriptors: brains, sheep, bovine spongiform encephalopathy, cattle, specimen handling, Scotland.

12. Barclay, G. Robin; Houston, E. Fiona; Halliday, Sue I.; Farquhar, Christine F.; Turner, Marc L. Comparative analysis of normal prion protein expression on human, rodent, and ruminant blood cells by using a panel of prion antibodies. Transfusion. 2002 May; 42(5): 517-26. ISSN: 0041-1132.
    Abstract: BACKGROUND: It is not known whether variant CJD can be transmitted within the human population by blood transfusion. The expression of normal cellular prion protein (PrPC) by different blood cell types may permit selective uptake and dissemination of infectivity. STUDY DESIGN AND METHODS: The normal distribution of PrPC on the major blood cell types of species known to be susceptible to natural or experimental transmissible spongiform encephalopathies was studied. Blood from healthy humans, mice, hamsters, cattle, and sheep was examined by flow cytometry by using a large panel of antibodies with different prion protein (PrP) epitope specificities to maximize the detection of PrP variants across species and cell type. RESULTS: PrP was detected on all major human blood cells types except eosinophils, but was not detected as ubiquitously or uniformly on major blood cell types of different animal species. CONCLUSION: Different animal species have unique patterns of expression of PrPC on blood cell types, with none equivalent to the human pattern. This needs to be considered when extrapolating from animal models of blood-borne transmissible spongiform encephalopathy infectivity, particularly in regard to the risk assessment of potential variant CJD spread within the human population. The relationship between PrP distribution and infectivity distribution in blood needs further investigation.
    Descriptors: NvCreuzfeldt-Jakob Disease, prion protein, major blood cells types, humans, C57BL mice hamsters, cattle, sheep, detection of PrP variants, comparison of human results to animals models.

13. Barnicle, D.A. Analysis by Western immunoblotting of differential enzymatic digestion of PrPSc to determine strain of agent. Research in Veterinary Science. April, 2002; 72 (Supplement A): 47. 56th Annual Conference of the Association of Veterinary Teachers and Research Workers on Current Topics in Veterinary Science, Scarborough, England, UK, March 25-27, 2002.
    NAL call no. 41.8 R312
    Descriptors: prion protein, PrPSc, strain typing, Western blot analysis.

14. Baron, Thierry. Identification of inter-species transmission of prion strains. Journal of Neuropathology and Experimental Neurology. 2002 May; 61(5): 377-83 ISSN: 0022-3069
    Journal URL: http://www.jneuropath.com/pt/re/jnen/home.htm/
    Abstract: The concern of the potential transmission of animal spongiform encephalopathies to humans, which arose as soon as the interspecies transmission of these diseases was recognized, has been reinforced with the emergence of bovine spongiform encephalopathy (BSE) in cattle. Recent experimental findings suggest that the infectious agent causing BSE in cattle can lead to the occurrence of a new form of Creutzfeldt-Jakob disease in humans. These findings help us understand how the transmission to humans of an animal disease may be recognized. This can involve an indirect approach through the analysis of neurodegeneration, either in the disease host, or more specifically, in genetically well-defined experimental hosts to which the disease can be transmitted. Recent experimental studies have also shown that the different molecular features of the abnormal form of the prion protein, which accumulates in the infected tissues, can provide important clues to the relationships between different spongiform encephalopathies. However, a better understanding of the molecular features associated with the specific pathogenic behavior of different strains is required. Complex relationships between the infectious agents involved in spongiform encephalopathies and the disease host can make the recognition of a link between animal prion strains and the human disease difficult to establish.
    Descriptors: cattle, bovine spongiform encephalopathy, prion proteins, molecular features, specific pathogenic behavior of different strains.

15. Beekes, M.; Kurth, R. BSE und Creutzfeldt-Jakob-Krankheit - Gesundheitspolitische Bedeutung fur die Bundesrepublik Deutschland und Europa. [BSE and Creutzfeldt-Jakob disease. Implication on health politics in Germany and Europe]. Deutsche Medizinische Wochenschrift 2002 Feb 15; 127(7): 335-40. ISSN: 0012-0472. In German
    NAL call no. 448.8 D48
    Descriptors: bovine spongiform encephalopathy, prevention, control, epidemiology, transmission, cats, cattle, sheep, Belgium, Germany, France, Europe, meat contamination.

16. Behrens, Axel; Aguzzi, Adriano. Small is not beautiful: Antagonizing functions for the prion protein PrPC and its homologue Dpl. Trends in Neurosciences. March, 2002; 25 (3): 150-154.
    DOI: 10.1016/S0166-2236(00)02089-0
    NAL call no. RC321 T74
    Descriptors: prion conformation variant, doppel protein, homology with PrPC, Dpl is dispensable for prion disease progression, prion biology.

17. Biedermann, Wolfgang; Lucker, Ernst; Hensel, Andreas. Detection of tissues of the central nervous system (CNS) as specified risk material (SRM) in meat products by means of gas chromatography-mass spectrometry (GC-MS). Berliner und Munchener Tierarztliche Wochenschrift 2002 Mar-Apr; 115(3-4): 131-3 ISSN: 0005-9366
    Abstract: Determination of specified risk material (SRM) in processed meat products was performed by quantification of brain specific fatty acids using gas chromatography-mass spectrometry (GC-MS). Results from SMP (internal standardised meat products) based analyses showed that absolute concentrations of CNS are correlated (r2 = > 0.97) with the contents of the CNS typical fatty acids docosahexaenoic acid (C 22:6), nervonic acid (C 24:1), lignoceric acid (C 24) and cerebronic acid (C 24oh). GC-MS detection limits were measured at 0.01% CNS. The cut off value was calculated at 0.39% (w/w) CNS in SMP. In a controlled blindfold experiment we were able to identify correctly all positive and negative SMP samples, respectively. Our results indicate that GC-MS based SRM detection may serve as a reference method for immunochemical and immunohistochemical determination of SRM in processed meat products.
    NAL call no. 41.8 B45
    Descriptors: neural tissue contamination, meat products analysis, immunochemical and immunohistochemical determination.

18. Biffiger, Karin; Zwald, Daniel; Kaufmann, Lukas; Briner, Alexandra; Nayki, Inci; Purro, Mario; Bottcher, Sigrid; Struckmeyer, Thomas; Schaller, Olivier; Meyer, Rudolf; Fatzer, Rosemarie; Zurbriggen, Andres; Stack, Mick; Moser, Markus; Oesch, Bruno; Kubler, Eric. Validation of a luminescence immunoassay for the detection of PrP(Sc) in brain homogenate. Journal of Virological Methods. 2002 Mar; 101(1-2): 79-84. ISSN: 0166-0934.
    Abstract: A luminescence immunoassay (LIA) was developed for the diagnosis of bovine spongiform encephalopathy (BSE) in brain tissue using two different monoclonal antibodies for capture and detection of the protease-resistant fragment of the pathological prion protein (PrP27-30). PrP27-30 currently represents the most reliable marker for the infectious particle (denominated prion) causing transmissible spongiform encephalopathies (TSEs). Internal and official validation studies of this assay are described using brain homogenates from ascertained BSE positive and negative cows. Using more than 300 positive and 1400 negative bovine or ovine samples, an excellent sensitivity and specificity of 100% were demonstrated. More than 1000-fold dilutions of a BSE positive homogenate still resulted in a clear positive signal. In combination with a simple homogenisation procedure for the preparation of the samples, this assay lends itself for large scale screening of cattle and sheep for TSEs using complete automation of the process.
    NAL call no. QR355.J6
    Descriptors: BSE, brain tissue analysis, cattle, diagnostic test, luminescense immunoassay, brain homogenates, screening of cattle and sheep for transmissible spongiform encephalopathies.

19. Bingham, Brian. New variant CJD-BSE (mad cow disease). The need for disposable ENT instruments. International Journal of Pediatric Otorhinolaryngology. 2002 Feb 25; 62(3): 203-6. ISSN: 0165-5876.
    Abstract: This paper outlines the development of Bovine Spongiform Encephalopathy (BSE) in the United Kingdom. The relationship between BSE and new variant Creutzfeldt-Jakob disease (vCJD) is considered and the risks of iatrogenic spread reviewed. The rationale for disposable surgical instruments in adenotonsillectomy to prevent iatrogenic spread is discussed.
    Descriptors: BSE, bovine spongiform encephalopathy, iatrogenic spread, surgical instruments, risks of transmission, UK.

20. Blattler, Thomas. Transmission of prion disease. APMIS. Acta Pathologica, Microbiologica, et Immunologica Scandinavica. 2002 Jan; 110(1): 71-8. ISSN: 0903-4641
    Abstract: The transmission of bovine spongiform encephalopathy to humans as variant Creutzfeldt-Jakob disease (vCJD) has focused public attention on how prion diseases are transmitted and how prions reach the brain after exposure. Prion diseases are characterised by transmissibility and neuropathological features of gliosis, neuronal loss and microscopic vacuoles, termed spongiosis. The principal component of prions is the glycoprotein PrP(Sc), which is a conformational modified isoform of the normal membrane protein PrP(C). How are prions transmitted and how do prions find their way once they have been ingested? Prion models in mouse and hamster point to lymphoreticular cells which support an early replication phase of prions before reaching the central nervous system via peripheral nerves. Whilst some key players seem to have been identified so far, the mechanisms of prion propagation to the brain are still not fully understood. Seemingly contradictory results have led to some confusion and have provoked discussion.
    NAL call no. QR1.A6
    Descriptors: BSE, spongiform encephalopathies, transmission, Nv Creutzfeldt-Jakob Disease, path to brain after exposure, mouse and hamster animal models, pmyphoreticular cells, peripheral nerve pathway, discussion of mechanism of prion propagation.

21. Bons, Noelle; Lehmann, Sylvain; Mestre, Frances-Nadine; Dormont, Dominique; Brown, Paul Brain and buffy coat transmission of bovine spongiform encephalopathy to the primate Microcebus murinus. Transfusion. May, 2002; 42 (5): 513-516.
    Descriptors: BSE, NvCreutzfeldt-Jacob Disease, secondary transmission, blood and blood products, macaque monkeys, Microcebus murinus, challenged with brain homogenate and buffy coat, transmission of infection.

22. Bons, Noelle; Lehmann, Sylvain; Nishida, Noriyuki; Mestre, Frances Nadine; Dormont, Dominique; Belli, Patrick; Delacourte, Andre; Grassi, Jacques; Brown, Paul. BSE infection of the small short-lived primate Microcebus murinus. Comptes Rendus Biologies. Janvier, 2002; 325 (1): 67-74.
    DOI: 10.1016/S1631-0691(02)01390-2
    NAL call no: Q2 C6
    Descriptors: Microcebus murinus (lemur) primates, disease susceptibility, intracerebrally/orally infected prion proteins, BSE /macaque-adapted BSE brain inoculates, hyperaggregated and paired-helical filaments-immunoreactive Tan proteins, beta42-amyloid plaques and astrogliosis, PrPres.

23. Boratynski, J.; Gorski, A. BSE: A consequence of cattle feeding with glycated molecules host-unknown? Medical Hypotheses. April, 2002; 58 (4): 276-278.
    Descriptors: pathogenesis, BSE, transmissible spongiform encephalopathies, scrapie, sheep, goats, cattle, disease origins, glycated proteins in feeds, high temperature glycation process.

24. Borchers, Kerstin. Transmissible Spongiforme Enzephalopathien (TSE): Alte Krankheiten mit neuer Brisanz. [Transmissible spongiform encephalopathies (TSE): old diseases with new explosive force]. Berliner und Munchener Tierarztliche Wochenschrift 2002 Mar-Apr; 115(3-4): 81-90 ISSN: 0005-9366. In German.
    Abstract: In view of the first 64 BSE cases (date: 11.5.01) in German cattle herds an overview on TSE and their similarities and differences regarding clinic, pathogenesis and pathology is given. The mechanism of the unconventional agent, an infectious protein (prion), is explained based on the prion model of Stanley Prusiner. The knowledge on transmission, incubation time, host specificity as well as resistance and immunity drawn from experimentally infected animals is discussed. Thus, after oral infection prions are transported by lymphocytes from the stomach-intestinal tract to the spleen. The way to the CNS is still unknown. The presumption for crossing the species barrier is twofold: first the prions of different species have to be biochemically homologous and a genetical disposition has to exist. This is the case for BSE and the new variant of Creutzfeldt-Jakob-Disease (vCJD). There is evidence that in Great Britain so far 97 (date: 30.3.01) young people acquired vCJD due to consumption of food that contained bovine risk material. Regarding the infectious prion dosis brain, spinal cord and lymphoid tissues are regarded to be most dangerous. The principle of the BSE-test, its evidence as well as steps for prevention and control of BSE are presented.
    NAL call no. 41.8 B45
    Descriptors: cattle, transmissible spongiform encephalopathies, comparisons, pathogenesis, pathology, prion proteins, incubation time, immunity, vCreutzfeldt-Jakob Disease, Germany.

25. Bosque, Patrick J.; Ryou, Chongsuk; Telling, Glenn; Peretz, David; Legname, Giuseppe; DeArmond, Stephen J.; Prusiner, Stanley B. Prions in skeletal muscle. Proceedings of the National Academy of Sciences of the United States of America. 2002 Mar 19; 99(6): 3812-7. ISSN: 0027-8424.
    Abstract: Considerable evidence argues that consumption of beef products from cattle infected with bovine spongiform encephalopathy (BSE) prions causes new variant Creutzfeldt-Jakob disease. In an effort to prevent new variant Creutzfeldt-Jakob disease, certain "specified offals," including neural and lymphatic tissues, thought to contain high titers of prions have been excluded from foods destined for human consumption [Phillips, N. A., Bridgeman, J. & Ferguson-Smith, M. (2000) in The BSE Inquiry (Stationery Office, London), Vol. 6, pp. 413-451]. Here we report that mouse skeletal muscle can propagate prions and accumulate substantial titers of these pathogens. We found both high prion titers and the disease-causing isoform of the prion protein (PrP(Sc)) in the skeletal muscle of wild-type mice inoculated with either the Me7 or Rocky Mountain Laboratory strain of murine prions. Particular muscles accumulated distinct levels of PrP(Sc), with the highest levels observed in muscle from the hind limb. To determine whether prions are produced or merely accumulate intramuscularly, we established transgenic mice expressing either mouse or Syrian hamster PrP exclusively in muscle. Inoculating these mice intramuscularly with prions resulted in the formation of high titers of nascent prions in muscle. In contrast, inoculating mice in which PrP expression was targeted to hepatocytes resulted in low prion titers. Our data demonstrate that factors in addition to the amount of PrP expressed determine the tropism of prions for certain tissues. That some muscles are intrinsically capable of accumulating substantial titers of prions is of particular concern. Because significant dietary exposure to prions might occur through the consumption of meat, even if it is largely free of neural and lymphatic tissue, a comprehensive effort to map the distribution of prions in the muscle of infected livestock is needed. Furthermore, muscle may provide a readily biopsied tissue from which to diagnose prion disease in asymptomatic animals and even humans.
    NAL call no. 500 N21P
    Descriptors: BSE, infected beef products, NvCreutzfeldt-Jacob disease, mouse model, skeletal muscle accumulations, PrPSc, Me7, Rocky Mountain Laboratory strain murine prions, possible diagnostic tissue for testing.

26. Bousset, Luc; Melki, Ronald. Similar and divergent features in mammalian and yeast prions. Microbes and Infection. Institut Pasteur 2002 Apr; 4(4): 461-9. ISSN: 1286-4579.
    Abstract: Mammalian transmissible spongiform encephalopathies are likely due to the propagation of an abnormal form of a constitutive protein instead of traditional genetic material (nucleic acids). Such infectious proteins, which are termed prions, exist in yeast. They are at the origin of a number of phenotypes that are inherited in a non-Mendelian manner. These prions are very useful to dissect the molecular events at the origin of this structure-based inheritance. The properties of mammalian and yeast prions are presented and compared. This review highlights a number of similarities and differences.
    NAL call no. QR180 M53
    Descriptors: TSEs, transmissible spongiform encephalopathies, infectious proteins, prions, yeast and mammalian prions compared, a review.

27. Boyce, Nell. The madness of the elk. US News and World Report. 2002 Mar 25; 132(9): 56. ISSN: 0041-5537.
    NAL call no. 280.8 Un33A
    Descriptors: animal diseases, etiology, wild deer, epidemiology, transmissible spongiform encephalopathy, BSE, USA.

28. Bradley, Ray. Bovine spongiform encephalopathy update. Polish Journal of Pathology Official Journal of the Polish Society of Pathologists 2002; 53(1): 7-16. ISSN: 1233-9687.
    Abstract: Bovine spongiform encephalopathy (BSE) is a zoonosis being the origin of variant Creutzfeldt-Jakob disease and an important cattle disease in its own right. Countries have been slow to learn the importance of protecting, not only their cattle populations, but also their human populations. Since 2000, several additional European countries have reported BSE in native-born stock and this has led to a concern about the BSE status of countries that have imported cattle and cattle products from infected countries. Extensive feed and offal bans and application of newly-developed, "Rapid" tests for prion protein in central nervous tissue of targeted, high-risk animals and slaughter cattle over 30 months old now provides the tools whereby the public are fully protected and BSE can be eradicated.
    Descriptors: BSE, NvCreutzfeldt-Jakob Disease, cattle, human health risks, prion protein testing in slaughtered animals, control, eradication.

29. Bren, Linda. FDA continues work to help prevent mad cow disease. FDA Consumer. 2002 May-Jun; 36(3): 31-2. ISSN: 0362-1332
    NAL call no. HD9000.9 U5A1
    Descriptors: BSE, bovine spongiform encephalopathy, transmission prevention and control, U.S. Food and Drug Administration, sentinel and surveillance, cattle, animal feeds, U.S.

30. Brenig, Bertram; Schutz, Ekkehard; Urnovitz, Howard. Zellulare Nucleinsauren im Serum und Plasma als neue diagnostische Werkzeuge. [Cellular nucleic acids in serum and plasma as new diagnostic tools]. Berliner und Munchener tierarztliche Wochenschrift 2002 Mar-Apr; 115(3-4): 122-4 ISSN: 0005-9366. In German.
    Abstract: Currently, the diagnosis of bovine spongiform encephalopathy is only possible in the brain stem of dead animals. Protease resistant prions are detected in the obex region of the brain stem. However, from a veterinary medical and agricultural point of view the development of an in vivo detection assay is of utmost importance. Because infectious prions are detectable relatively late in the central nervous system during an infection, efforts are made searching for surrogate markers. Besides neuronal proteins that are released into the liquor and blood during neurodegenerative processes or other neuronal diseases, cellular nucleic acids circulating in the plasma or serum are an absolutely new approach for the detection of infectious diseases.
    NAL call no. 41.8 B45
    Descriptors: BSE, bovine spongiform encephalopathy, vivo detection assay, new approach, cellular nucleic acids in plasma or serum.

31. Breslin, P. D.; Bassett, H. F.; McElroy, M.C.; Markey, B. K.; Weavers, E. Brainstem cell populations in BSE-affected and clinically normal cattle. Research in Veterinary Science. April 2002; 72 (Supplement A): 46. 56th Annual Conference of the Association of Veterinary Teachers and Research Workers on Current Topics in Veterinary Science, Scarborough, England, UK, March 25-27, 2002
    NAL call no. 41.8 R312
    Descriptors: cattle, bovine spongiform encephalopathy, brainstem cells, comparison study, diseased and normal animals.

32. Brown, David R Mayhem of the multiple mechanisms: modelling neurodegeneration in prion disease. Journal of Neurochemistry. 2002 Jul; 82(2): 209-15 ISSN: 0022-3042.
    Abstract: This review examines recent attempts to advance the understanding of the mechanism by which neurones die in prion disease. Prion diseases or transmissible spongiform encephalopathies are characterized by the conversion of a normal glycoprotein, the prion protein, to a protease-resistant form that is suggested to be both the infectious agent and the cause of the rapid neurodegeneration in the disease. Death of the patient results from this widespread neuronal loss. Thus understanding the mechanism by which the abnormal form of the prion protein causes neuronal death might lead to treatments that would prevent the life-threatening nature of these diseases.
    NAL call no. QP351.J6
    Descriptors: prion diseases, neuronal death patterns, animal models, physiopathology, transmissible spongiform encephalopathies.

33. Brown, David R. Molecular advances in understanding inherited prion diseases. Molecular Neurobiology. June, 2002; 25 (3): 287-302.
    NAL call no. QP365.2 M64
    Descriptors: BSE, bovine spongiform encephalopathy, variant Creutzfeldt Jakob disease, CJD.

34. Brown, Paul. Drug therapy in human and experimental transmissible spongiform encephalopathy. Neurology. 2002 Jun 25; 58(12): 1720-5. ISSN: 0028-3878.
    Abstract: During the past 30 years, over 60 different chemical compounds have been used to treat experimental animals infected with transmissible spongiform encephalopathies (TSE), including a wide variety of anti-infectious agents, immunomodulating drugs, and chemicals interacting with the lympho-reticular system. Some compounds achieved a prolongation of the incubation period, but this effect decreased or disappeared when they were administered at or near the onset of symptomatic disease. Recent in vitro and tissue culture studies support earlier speculation about the importance of a chemical structure containing both water-soluble and lipid-soluble components, evidently as a means of interaction with the misfolded membrane-bound 'prion' protein. A number of compounds shown to eliminate the protein (or infectivity) in TSE-infected tissue cultures are the subject of ongoing studies in animals, and are under consideration for human drug trials. As with other recalcitrant infections, combinations of drugs with different modes of action are likely to be necessary for any effective therapy. Also, very recent work in developing antibodies that can neutralize in vitro infection (and, in conjunction with genetic engineering, in vivo infection) has renewed interest in the strategies of both active and passive immunization.
    Descriptors: drugs, treatments, TSEs, anti-infectious agents, immunomodulation drugs, chemicals, experimental animals, prion diseases, prion proteins.

35. Bruce, Moira E.; Boyle, Aileen; Cousens, Simon; McConnell, Irene; Foster, James; Goldmann, Wilfred; Fraser, Hugh Strain characterization of natural sheep scrapie and comparison with BSE. Journal of General Virology. 2002 Mar; 83(Pt 3): 695-704 ISSN: 0022-1317.
    Abstract: Scrapie was transmitted to mice from ten sheep, collected in the UK between 1985 and 1994. As in previous natural scrapie transmissions, the results varied between scrapie sources in terms of the incidence of disease, incubation periods and neuropathology in challenged mice. This contrasted with the uniformity seen in transmissions of BSE to mice. The scrapie and BSE isolates were characterized further by serial passage in mice. Different TSE strains were isolated from each source according to the Sinc or PrP genotype of the mouse used for passage. The same two mouse-passaged strains, 301C and 301V, were isolated from each of three BSE sources. Despite the variation seen in the primary transmissions of scrapie, relatively few mouse-passaged scrapie strains were isolated and these were distinct from the BSE-derived strains. The ME7 scrapie strain, which has often been isolated from independent sheep sources in the past, was identified in isolates from four of the sheep. However, a new distinct strain, 221C, was derived from a further four scrapie sheep. These results suggest that there is agent strain variation in natural scrapie in sheep and that the spectrum of strains present may have changed over the last 20 years. The tested sample is too small to come to any conclusions about whether the BSE strain is present in sheep, but the study provides a framework for further more extensive studies.
    NAL call no. QR360.A1J6
    Descriptors: scrapie, transmitted to mice, BSE isolates, serial passage in mice, ME7 strain, 221C strain, strain evolution.

36. Brufau, J.; McCartney, E. 'Feed scares': where is Europe leading? Feed International. 2002, 23: 2, 6-8.
    Descriptors: animal feeds, transmissible spongiform encephalopathies, livestock animals, epidemiology, control, Europe.

37. Bucciantini, Monica; Giannoni, Elisa; Chiti, Fabrizio; Baroni, Fabiana; Formigli, Lucia; Zurdo, Jesus; Taddei, Niccolo; Ramponi, Giampietro; Dobson, Christopher M.; Stefani, Massimo. Inherent toxicity of aggregates implies a common mechanism for protein misfolding diseases. Nature. April 4, 2002; 416 (6880): 507-11. ISSN: 0028-0836
    DOI: 10.1038/416507a
    Abstract: A range of human degenerative conditions, including Alzheimer's disease, light-chain amyloidosis and the spongiform encephalopathies, is associated with the deposition in tissue of proteinaceous aggregates known as amyloid fibrils or plaques. It has been shown previously that fibrillar aggregates that are closely similar to those associated with clinical amyloidoses can be formed in vitro from proteins not connected with these diseases, including the SH3 domain from bovine phosphatidyl-inositol-3'-kinase and the amino-terminal domain of the Escherichia coli HypF protein. Here we show that species formed early in the aggregation of these non-disease-associated proteins can be inherently highly cytotoxic. This finding provides added evidence that avoidance of protein aggregation is crucial for the preservation of biological function and suggests common features in the origins of this family of protein deposition diseases.
    NAL call no. 472 N21
    Descriptors: spongiform encephalopathies, proteinaceous aggregates, fibrillar aggregates, bovine phosphatidylinositol-3-kinase chemistry. PC12 cells, rats.

38. Budka, Herbert; Dormont, Dominique; Kretzschmar, Hans; Pocchiari, Maurizio; van-Duijn, Cornelia.. BSE and variant Creutzfeldt-Jakob disease: never say never. Acta Neuropathologica 2002 Jun; 103(6): 627-8 ISSN: 0001-6322
    Descriptors: bovine spongiform encephalopathy, NvCJD, prion pathogenicity, epidemiology, animal models, transmission and control.

39. Busk, N.; Vaughan, K.; Watkins, R.; Hawkins-S.A.C. Review and refinement of clinical monitoring methods for TSE mouse bioassay/strain typing studies. Research in Veterinary Science. April, 2002; 72 (Supplement A): 46-47. 56th Annual Conference of the Association of Veterinary Teachers and Research Workers on Current Topics in Veterinary Science, Scarborough, England, UK, March 25-27, 2002.
    NAL call no. 41.8 R312
    Descriptors: transmissible spongiform encephalopathies, mouse model bioassay, strain typing, prions.

40. Cai, Kang; Miller, Jeanette L.C.; Stenland, Christopher J.; Gilligan, Kevin J.; Hartwell, Randal C.; Terry, Jarrett C.; Evans-Storms, Rosemary B.; Rubenstein, Richard; Petteway, Stephen R. Jr; Lee, Douglas C. Solvent-dependent precipitation of prion protein. Biochimica et Biophysica Acta - Protein Structure and Molecular Enzymology. 20 May, 2002; 1597 (1): 28-35. ISSN: 0006-3002.
    NAL call no. 381 B522
    Descriptors: misfolded isoform, prion protein, PrPSc, solvents, physiological buffer, pH effects, salt, ethanol concentration.

41. Carlson, George A. Postexposure prophylaxis against transmissible spongiform encephalopathies: CpG oligodeoxynucleotides in mice. Lancet. 2002 Jul 20; 360(9328): 184. ISSN: 0140-6736.
    NAL call no. 449.8 L22
    Descriptors: cattle, adjuvants, immunologic therapeutic use, BSE, control, mouse models, oligodeoxyribonucleotides.

42. Carruthers, Jean; Carruthers, Alastair, Mad cows, prions, and wrinkles. Archives of Dermatology. 2002 May; 138(5): 667-70 ISSN: 0003-987X.
    NAL call no. 448.8 AR242
    Descriptors: BSE transmission risks, collagen, prion physiology, prion disease prevention and control, cosmetic formulations, injections, intradermal.

43. Chamberland, Mary E. Emerging infectious agents: do they pose a risk to the safety of transfused blood and blood products? Clinical Infectious Diseases, Official Publication of the Infectious Diseases Society of America. 2002 Mar 15; 34(6): 797-805. ISSN: 1537-6591.
    Abstract: The blood supply is safer than it has been at any other time in recent history, and, in the context of other health care-related adverse events, the risks associated with blood transfusion are extremely small. The current high level of safety is the result of successive refinements and improvements in how blood is collected, tested, processed, and transfused; nonetheless, blood and plasma products remain vulnerable to newly identified or reemerging infections. In recent years, numerous infectious agents-including several newly discovered hepatitis viruses, the agents of transmissible spongiform encephalopathies, and tickborne pathogens-have been identified as potential threats to the safety of blood and plasma. Continued vigilance is critical to protect the blood supply from known pathogens and to monitor for the emergence of new infectious agents. Recent terrorist activities in the United States add new considerations to maintaining the safety and supply of blood. Education of clinicians and patients regarding the benefits and risks associated with the judicious use of blood and blood products can assist in informed decision making.
    NAL call no. RC111 R4
    Descriptors: blood supply, safety risks, transmissible spongiform encephalopathies, recommendations for decision making.

44. Chaplin, M.J.; Barlow, N.; Ryder, S.; Simmons, M.M.; Spencer, Y.; Hughes, R.; Stack, M. J. Evaluation of the effects of controlled autolysis on the immunodetection of PrPSc by immunoblotting and immunohistochemistry from natural cases of scrapie and BSE. Research in Veterinary Science. February, 2002; 72 (1): 37-43.
    NAL call no. 41.8 R312
    Descriptors: sheep, cattle, scrapie, BSE, histopathological examination, medulla brain exam, Western immunoblotting for detection of PrPSc, Prionics diagnostic test, routine immunohistochemical technique.

45. Cochrane, N. Pressures for change in Eastern Europe's livestock sectors. Agricultural Outlook. 2002, No.288, 17-20.
    NAL call no. aHD1751.A42
    Descriptors: Central Europe, disease control, transmissible spongiform encephalopathy, livestock testing, trade, disease prevention and control.

46. Cooper, J.E. Diagnostic pathology of selected diseases in wildlife. Revue Scientifique et Technique Office International des Epizooties. April, 2002; 21 (1): 77-89.
    URL: www.oie.int/eng/publicat/rt/2101/A_R2115.htm
    NAL call no. SF781 R4
    Descriptors: wildlife, detection, management, infectious disease, field diagnosis, sampling, principles, diagnostic pathology, mycobacteriosis, Rift Valley fever, rabies, spongiform encephalopathies, morbillivirus and poxvirus infections, viral encephalitides, West Nile virus infection and chytridiomycosis.

47. Croes, E.A.; van Gool, W.A.; Jansen, G. H.; van Duijn, C. M. Ziekte van Creutzfeldt-Jakob: diagnostiek, incidentie, preventie en behandeling. [Creutzfeldt-Jakob disease: diagnosis, incidence, prevention and treatment]. Nederlands Tijdschrift voor Geneeskunde 2002 Apr 20; 146(16): 750-4. ISSN: 0028-2162. In Dutch.
    Abstract: Creutzfeldt-Jakob disease (CJD) is a rare, neurodegenerative disorder belonging to the spongiform encephalopathies. A variant form (vCJD) is most likely the result of infection with the agent that causes bovine spongiform encephalopathy (BSE). Diagnostic information can be obtained by EEG, testing cerebrospinal fluid for the presence of the 14-3-3 protein, MRI, brain biopsy, tonsil biopsy, and postmortem brain examination. Some tests, such as MRI and postmortem brain examination, can be used to distinguish between CJD and vCJD. Pathological prions in a tonsil biopsy are only found with vCJD. In the Netherlands, there are four known cases of iatrogenic CJD. On the basis of certain exposure to BSE via the food chain, cases of vCJD are also to be expected. Chloropromazine and mepacrine are known to inhibit the formation of pathological prion conformations, but clinical trials have not yet been carried out.
    Descriptors: NvCJD, bovine spongiform encephalopathy, BSE, diagnosis of CJD and NvCJD, humans, iatrogenic disease, MRI, brain biopsy, tonsil biopsy, postmortem brain examination, chloropromazine, mepacrine, treatment.

48. Dalsgaard, Niels Jorn. Prion diseases. An overview. APMIS Acta Pathologica, Microbiologica, et Immunologica Scandinavica. 2002 Jan; 110(1): 3-13. ISSN: 0903-4641.
    Abstract: Prion disease is the new designation of a group of spongiform encephalopathies, all invariably fatal, which show similar clinical and neuropathological changes. They comprise a range of distinct diseases in both animals and man, and spontaneous, hereditary and transmissible forms are recognized. Until the sudden occurrence in the mid-1980s of an epizootic of a formerly unknown disease, popularly named 'mad cow disease', in cattle in the UK, very little attention had been paid to these rather obscure diseases. Concurrently it was asserted that the disease-causing agent appeared to be a ubiquitous mammalian brain constituent, and the disease mechanism a conformational change of its structure. These events have not only led to a new understanding of these extraordinary diseases, but have also provided insight into both neurodegeneration and disease mechanisms at the molecular level. Moreover, in 1997 the prion concept earned its originator the second Nobel price for medicine within this scientific field. In this introduction and overview of prion diseases, historical and philosophical perspectives are presented along with descriptions of the diseases in both animals and man. Epidemiology, genetics and transmissibility are also covered.
    NAL call no. QR1.A6
    Descriptors: spongiform encephalopathies, BSE, bovine spongiform encephalopathies, neurodegeneration, disease mechanisms, prions, overview, history, descriptions of diseases, epidemiology, genetics, transmissibility, US, UK.

49. Daly, D. J.; Prendergast, D. M.; Sheridan, J. J.; Blair, I.S.; McDowell, D.A. Use of a marker organism to model the spread of central nervous system tissue in cattle and the abattoir environment during commercial stunning and carcass dressing. Applied and Environmental Microbiology. 2002 Feb; 68(2): 791-8. ISSN: 0099-2240.
    Abstract: Due to concerns about a link between variant Creutzfeldt-Jakob disease in humans and similar prion protein-induced disease in cattle, i.e., bovine spongiform encephalopathy (BSE), strict controls are in place to exclude BSE-positive animals and/or specified risk materials including bovine central nervous system (CNS) tissue from the human food chain. However, current slaughter practice, using captive bolt guns, may induce disruption of brain tissues and mobilize CNS tissues into the bovine circulatory system, leading to the dispersion of CNS tissues (including prion proteins) throughout the derived carcass. This project used a marker (antibiotic-resistant) strain of Pseudomonas fluorescens to model the effects of commercial captive bolt stunning procedures on the movement of mobilized CNS material within slaughtered animals and the abattoir environment. The marker organism, introduced by injection through the bolt entry aperture or directly using a cartridge-fired captive bolt, was detected in the slaughter environment immediately after stunning and in the abattoir environment at each subsequent stage of the slaughter-dressing process. The marker organism was also detected on the hands of operatives; on slaughter equipment; and in samples of blood, organs, and musculature of inoculated animals. There were no significant differences between the results obtained by the two inoculation methods (P < 0.05). This study demonstrates that material present in, or introduced into, the CNS of cattle during commercial captive bolt stunning may become widely dispersed across the many animate and inanimate elements of the slaughter-dressing environment and within derived carcasses including meat entering the human food chain.
    NAL call 448.3 AP5
    Descriptors: NvCreutzfeldt-Jakob Disease, BSE, bovine spongiform encephalopathy, cattle, control and prevention of prion disease, slaughter, Pseutomonas fluorescens to model captive bolt stunning, facility monitoring, carcass contamination.

50. Dealler, Steve. vCJD: the epidemic that never was. Possibility of BSE being cause of variant CJD is indeed biologically plausible. BMJ Clinical Research Ed 2002 Jul 13; 325(7355): 102. ISSN: 1468-5833.
    Descriptors: cattle, bovine spongiform encephalopathy, NvCreutzfeldt-Jakob Syndrome transmission, contaminated meat, public health risks.

51. Debeer, Sabine O. S.; Baron, Thierry G.M.; Bencsik, Anna A. Transmissible spongiform encephalopathy diagnosis using PrPsc immunohistochemistry on fixed but previously frozen brain samples. Journal of Histochemistry and Cytochemistry. The official journal of the Histochemistry Society 2002 May; 50(5): 611-6. ISSN: 0022-1554.
    URL: www.jhc.org/cgi/content/abstract/50/5/611
    Abstract: The histological diagnosis of transmissible spongiform encephalopathies (TSEs), such as scrapie and bovine spongiform encephalopathy (BSE), relies on identification in the brain of spongiosis, gliosis, and neuron loss without inflammatory lesions. Because of its sensitivity, immunohistochemistry of abnormal prion protein (PrPsc) is of great help in this diagnosis and can be used on its own or complementary to the biochemical detection of PrPsc. However, in some cases no formalin-fixed material is available, rendering its use as a complementary method impossible. For that purpose, we studied the possibility of detecting PrPsc immunohistochemically in fixed brain samples that had been previously frozen and used for Western blotting analysis. We compared freshly and fixed-frozen brain samples originating from the same sheep, either affected or unaffected with scrapie. We also studied fixed-frozen brain samples from scrapie-affected goats and from cows showing BSE. We showed that in all the species tested, despite damage to the histological structures, PrPsc was still detectable in the fixed-frozen brain sections without unspecific background staining. Notwithstanding the limited number of cases thus far analyzed, we have already demonstrated the possibility of using PrPsc immunohistochemistry on fixed-frozen brain samples with very good efficacy, thus rendering possible its use for diagnostic purposes in TSEs.
    NAL call no. 381 J8222
    Descriptors: scrapie, BSE, immunohistochemistry of abnormal prion protein, PrPsc, diagnosis, compared fresh and frozen brain tissue, diagnostic method.

52. Dedet,V. BSE screeningsprover pa levende dyr: tre vaesentlige eksempler pa videnskabelige undersogelser. [BSE screening for live animals. Three good examples of scientific experiments.] Dansk Veterinaertidsskrift. 2002, 85: 1, 18-19. In Danish.
    NAL call no. 41.9 D23
    Descriptors: cattle, sheep, bovine spongiform encephalopathy, diagnostic methods.

53. Delgado-Hachmeister, J.E.; Rangel-Frausto, M.S.; Ponce de Leon, S; de Leon, S. Ponce. Encefalopatias espongiformes transmisibles. [Transmissible spongiform encephalopathies.] Salud Publica de Mexico. 2002, 44: 1, 69-75; 22 ref. In Spanish with an English summary.
    Descriptors: TSE, public health concerns, Mexico.

54. Dickmeiss, Ebbe; Gerstoft, Jan. Blood infectivity in transmissible spongiform encephalopathies. APMIS. Acta Pathologica, Microbiologica, et Immunologica Scandinavica 2002 Jan; 110(1): 99-103 ISSN: 0903-4641.
    Abstract: Blood infectivity in transmissible spongiform encephalopathies (TSE) is reviewed with special emphasis on transmission by blood transfusion in human beings. It is concluded that transmission by transfusion seems biologically plausible as regards variant Creutzfeld-Jakob Disease (vCJD), albeit present knowledge suggests that it is extremely uncommon. Precautionary measures against the putative risk of vCJD transmission by blood transfusion are discussed.
    NAL call no. QR1.A6
    Descriptors: blood products, transmissibility concerns, transmissible spongiform encephalopathies, emphais on humans, NvCreutzfeldt-Jakob Disease, risks.

55. Docampo, Roberto. New and re-emerging infectious diseases. Trends in Parasitology. August, 2002; 18 (8): 334-336. The 5th Annual Conference on New and Re-emerging Infectious Diseases, Urbana-Champaign, IL, USA, April 18-19, 2002.
    DOI: 10.1016/S1471-4922(02)02351-6
    NAL call no. QL757 P374
    Descriptors: transmissible spongiform encephalopathies, various diseases are addressed, zoonotic and animal diseases.

56. Dodd, Roger Y.; Busch, Michael P. Animal models of bovine spongiform encephalopathy and vCJD infectivity in blood: two swallows do not a summer make. Transfusion. 2002 May; 42(5): 509-12. ISSN: 0041-1132.
    Descriptors: Creutzfeldt Jakob Syndrome, transmission, BSE, CJD, bovine spongiform spongiform encephalopathy, animals models, blood transfusion research, lemur, Macaca fascicularis, phosphoprotein-phasphatase analysis, sheep, species specificity.

57. Doherr, M.G. Aetiologie, Uebertragung und epidemiologische Situation von BSE in Europa. [Etiology, transmission and epidemiological situation of BSE in Europe.] Praktische Tierarzt 2002 vol. 83, no. 2, pp. 156-161. In German.
    NAL call no. 41.8 P882
    Descriptors: BSE, European status, epidemiology, etiology, public health concerns, transmissible spongiform encephalopathies.

58. Doherr, M.G.; Hett, A.R.; Cohen, C.H.; Fatzer, R.; Rufenacht, J.; Zurbriggen, A.; Heim, D. Trends in prevalence of BSE in Switzerland based on fallen stock and slaughter surveillance. Veterinary Record. London : The British Veterinary Association. Mar 16, 2002. v. 150 (11) p. 347-348. ISSN: 0042-4900
    NAL call no. 41.8 V641
    Descriptors: cattle, BSE, bovine spongiform encephalopathy, disease prevalence, trends, disease surveys, Switzerland.

59. Domingo, Jose L. Lack of experimental studies on human transmission of BSE in relation with the consumption of specified risk materials (SRM): The case of the milk. Preventive Medicine. June, 2002; 34 (6): 655-656.
    DOI: 10.1006/pmed.2002.1027
    NAL call no. RA421 P684
    Descriptors: BSE, bovine spongiform encephalopathy, transmissible spongiform encephalopathy, public health risks, animal derived foods, milk, research recommendations.

60. Eaton, S.L.; Foster, J. D.; Hunter, N. Follicular dendritic cell involvement in ovine scrapie. Research in Veterinary Science. April, 2002; 72 (Supplement A): 44. 56th Annual Conference of the Association of Veterinary Teachers and Research Workers on Current Topics in Veterinary Science, Scarborough, England, UK, March 25-27, 2002.
    NAL call no. 41.88 R312
    Descriptors: sheep, scrapie, prion disease, effects on follicular dendritic cells.

61. Eberl, Heike; Glockshuber, Rudi. Folding and intrinsic stability of deletion variants of PrP(121-231), the folded C-terminal domain of the prion protein. Biophysical Chemistry. 2 May, 2002; 96 (2-3): 293-303.
    DOI: 10.1016/S0301-4622(02)00015-7
    NAL call no. QP1.B5
    Descriptors: transmissible spongiform encephalopathies, prions, PrPSc, PrPC structure, deletion variants of C terminal PrPC domain, tertiary structure context, conformation of the segment comprising alpha-helix 2 and 3 in the solution structure of recombinant PrP.

62. Farrugia, Albert. Laboratory practice and studies of bovine spongiform encephalopathy. Lancet. 2002 Mar 23; 359(9311): 1067-8. ISSN: 0140-6736.
    NAL call no. 448.4 L22
    Descriptors: bovine spongiform encephalopathy transmission, laboratory standards, cattle, sheep.

63. Fatzer, R.; Vandevelde, M.; Gottstein, B. Cerebral taeniid oncospheral lesions in two BSE suspects. Veterinary Record. London : The British Veterinary Association. Jan 12, 2002. v. 150 (2) p. 46-47. ISSN: 0042-4900.
    NAL call no. 41.8 V641
    Descriptors: cattle, brain lesions, oncospheres, symptoms, BSE, bovine spongiform encephalopathy, histopathology, Taenia saginata, case report, Switzerland.

64. Ferguson, N.M.; Ghani, A.C.; Donnelly, C.A.; Hagenaars, T.J.; Anderson, R.M. Estimating the human health risk from possible BSE infection of the British sheep flock. Nature. London : Macmillan Magazines Ltd. Jan 24, 2002. v. 415 (6870) p. 420-424. ISSN: 0028-0836
    DOI: 10.1038/nature709
    Abstract: Following the controversial failure of a recent study and the small numbers of animals yet screened for infection, it remains uncertain whether bovine spongiform encephalopathy (BSE) was transmitted to sheep in the past via feed supplements and whether it is still present. Well grounded mathematical and statistical models are therefore essential to integrate the limited and disparate data, to explore uncertainty, and to define data-collection priorities. We analysed the implications of different scenarios of BSE spread in sheep for relative human exposure levels and variant Creutzfeldt-Jakob disease (vCJD) incidence. Here we show that, if BSE entered the sheep population and a degree of transmission occurred, then ongoing public health risks from ovine BSE are likely to be greater than those from cattle, but that any such risk could be reduced by up to 90% through additional restrictions on sheep products entering the food supply. Extending the analysis to consider absolute risk, we estimate the 95% confidence interval for future vCJD mortality to be 50 to 50,000 human deaths considering exposure to bovine BSE alone, with the upper bound increasing to 150,000 once we include exposure from the worst-case ovine BSE scenario examined.
    NAL call no. 472 N21
    Descriptors: cattle, BSE, bovine spongiform encephalopathy, infections, sheep, disease transmission. human diseases, zoonosis, public health, NvCreutzfeldt Jakob Disease, ovine BSE, statistical models, UK.

65. Ferguson-Smith, Malcolm A. Reaction to the emergence of BSE in the UK: What was done and what perhaps might have been done better. Comptes Rendus Biologies. Janvier, 2002; 325 (1): 25-26.
    DOI: 10.1016/S1631-0691(02)01395-1
    NAL call no. Q2 C6
    Descriptors: BSE, bovine spongiform encephalopathy, the UK epidemic, governmental actions, historical perspectives.

66. Fernie, K. Heat/hydroxide combinations as novel decontamination methods for surgical instruments. Research in Veterinary Science. April, 2002; 72 (Supplement A): 43-44. 56th Annual Conference of the Association of Veterinary Teachers and Research Workers on Current Topics in Veterinary Science, Scarborough, England, UK, March 25-27, 2002
    NAL call no. 41.8 R312
    Descriptors: transmissible spongiform encephalopies, prions, potential contamination of surgical instruments, decontamination methods.

67. Foster, J.D.; Parnham, D.W.; Hunter, N.; Bruce, M. Distribution of the prion protein in sheep terminally affected with BSE following experimental oral transmission. Research in Veterinary Science. April, 2002; 72 (Supplement A): 45. 56th Annual Conference of the Association of Veterinary Teachers and Research Workers on Current Topics in Veterinary Science, Scarborough, England, UK, March 25-27, 2002
    NAL call no. 41.8 R312
    Descriptors: sheep, BSE, bovine spongiform encephalopathy, prion proteins, experimental infection via oral route, distribution in the body.

68. Fraser, J.R. What is the basis of transmissible spongiform encephalopathy induced neurodegeneration and can it be repaired? Neuropathology and Applied Neurobiology 2002 Feb; 28(1): 1-11. ISSN: 0305-1846.
    Abstract: Once an animal becomes infected with a prion disease, or transmissible spongiform encephalopathy (TSE), the progression of infection is relentless and inevitably fatal, although often with such prolonged incubation periods that an alternative cause of death can intervene. Infection has been compared to 'setting a clock' which then runs inexorably as the disease spreads, usually through the lymphoreticular system and then via peripheral nerves to the central nervous system (CNS), although the mechanism controlling the protracted progression is not known. Clinical disease develops as characteristic degenerative changes in the CNS progress, but the molecular basis for this pathology is not clear, particularly the relationship between the deposition of abnormal PrP and neuronal dysfunction. Recent research has identified several means of slowing (if not stopping) the clock when infection has not yet reached the CNS; although the potential for later stage therapies seems limited, neuroprotective strategies which have been shown to be effective in other neurodegenerative conditions may also ameliorate TSE induced CNS pathology. This review focuses on our current knowledge of the key events following infection of the CNS and the opportunities for intervention once the CNS has become infected.
    Descriptors: prion disease, TSE, review of current knowledge of infection, possible opportunities for intervention of disease process.

69. Frey, Jacques. Bovine spongiform encephalopathy: Are the cows mad or full of carbohydrates? Clinical Chemistry and Laboratory Medicine. February, 2002; 40 (2): 101-103.
    Descriptors: dairy cows, diet, dysregulation of carbohydrate metabolism, neurodegenerative disorders, BSE, origin, prion proteins.

70. Gargani, G. Le encefalopatie spongiformi trasmissibili. Storia, epidemiologia, ipotesi eziologiche. [Transmissible spongiform encephalopathies. History, epidemiology, etiological, hyphotheses]. Minerva Medica 2002 Feb; 93(1): 59-73. ISSN:0026-4806. In Italian.
    Abstract: The history of transmissible spongiform encephalopathies is shortly reviewed beginning with the Westminster parliament act in the year 1755 up to the description in 1996 of the variant of the Creutzfeldt-Jakob disease, transmitted from cattle to man by alimentary route. The epidemiological patterns of encephalopathies of the various animal species and of the four encephalopathies up to date reported in man are shortly described: Creutzfeldt-Jakob disease, Kuru, Gerstmann-Straussler-Scheinker disease, Fatal Familial Insomnia. Etiological hypotheses are discussed until the identification of Prions: PrPcell, on the surface of normal cells, PrPscr in the brain of humans and animals dead for these diseases. The strains of the PrPscr are described on the basis of some characters observed through the passages in rodents and of molecular pattern. The possible future epidemiological evolution of the vCJD is also discussed.
    Descriptors: TSEs, historical review, 1755-1996, NvCreutzfeldt-Jakob Disease, cattle, man, epidemiology, Creutzfeldt-Jakob disease, Kuru, Gerstmann-Straussler-Scheinker disease, Fatal Familial Insomnia, PrPscr strains.

71. Ghani, Azra C. The epidemiology of variant Creutzfeldt-Jakob disease in Europe. Microbes and Infection. 2002 Mar; 4(3): 385-93.ISSN: 1286-4579.
    Abstract: Variant Creutzfeldt-Jakob disease is one of a family of neurodegenerative diseases, first diagnosed in 1996. Scientific evidence strongly supports the hypothesis that it is acquired through consumption of bovine spongiform encephalopathy-infected meat. The majority of cases have been diagnosed in the UK in young individuals, with an excess of cases in the north and a significant cluster of cases in Leicestershire. Many uncertainties in its biology and epidemiology, in particular the length of the incubation period, make predictions of any future epidemic difficult. Studies are currently under way to obtain more precise estimates of the prevalence of asymptomatic infection through testing tonsil and appendix tissues for the abnormal prion protein.
    NAL call no. QR180 M53
    Descriptors: NvCreutzfeldt-Jakob Disease, bovine spongiform infected meat consumption, human cases, young, biology, epidemiology, incubation period, estimates of asymptomatic infection.

72. Ghani, Azra C.; Donnelly, Christl A.; Ferguson, Neil M..; Anderson, Roy M. The transmission dynamics of BSE and vCJD. Comptes Rendus Biologies. Janvier, 2002; 325 (1): 37-47.
    DOI: 10.1016/S1631-0691(02)01389-6
    NAL call no. Q2 C6
    Descriptors: BSE, cattle, NvCreutzfeldt-Jakob disease, epidemiological models, transmission dynamics, case projections, Great Britian, Northern Ireland, Portugal, France.

73. Gray, George; Kreindel, Silvia; Ropeik, David. Mad cow disease risk in the United States. Does perceived threat overshadow true likelihood of occurrence? Postgraduate Medicine. 2002 Feb; 111(2): 13-4,16. ISSN: 0032-5481.
    Descriptors: BSE, bovine spongiform encephalopathy, animal feeds, epidemiology, transmission, NvCreutzfeldt-Jakob Disease, public health risk factors.

74. Groschup, Martin H.; Stolze, Anne. BSE- und Scrapie-Diagnostik in Deutschland. [BSE and scrapie diagnosis in Germany]. Berliner und Munchener Tierarztliche Wochenschrift 2002 Mar-Apr; 115(3-4): 106-10 ISSN: 0005-9366. In German.
    Abstract: The detection of pathological prion protein is considered as pathognomonic for the diagnosis of transmissible spongiform encephalopathies. According to the EU regulations cattle older than 30 months of age (Germany 24 months) and slaughtered for human consumption must be tested by using BSE rapid tests. Likewise must be fallen stock and clinically affected animals. This article gives an overview over the diagnostic hierarchy and organization of the diagnostic system for BSE and scrapie in Germany. All suspect cases found by rapid testing are reinvestigated and clarified by the National reference laboratory for these diseases which is part of the recently founded Institute of Novel and Emerging Infectious Diseases at the Federal Research Centre for Virus Diseases of Animals located on the isle of Riems. Until the end of 2001 130 BSE cases were confirmed out of 230 submissions.
    NAL call no. 41.8 B45
    Descriptors: cattle, slaughtered animals, BSE and scrapie rapid tests, diagnostic hierarchy, Germany.

75. Gunn, H. M.; Lynch, D.; Sheridan, H.; Costelloe, A.; Weavers, E.; McElroy, M.; Cooney, J.; Sammin, D.; Good, M.; O' Sullivan, M.; Caulfield, L.; Kennedy, S.; Smyth, K.; Carr, M.; Sheridan, J.; Casey, V.; Curley, L.; Lynch, M.; Gleeson, B. Alteration in age profile of BSE cases in Ireland. Irish Veterinary Journal. 2002, 55: 2, 84-85.
    NAL call no. 41.8 IR4
    Descriptors: bovine spongiform encephalopathy, age comparison, disease incidence, Irish Republic, UK.

76. Harrison, M. The dairy industry looks to the future. Food Science and Technology. 2002, 16: 1, 24-30.

77. Heppner, F. L.; Aguzzi, A. Immunitat gegen Prionen? [Immunity against prions?] Deutsche Medizinische Wochenschrift 2002 Feb 15; 127(7): 328-30. ISSN: 0012-0472. In German.
    NAL call no. 448.8 D48
    Descriptors: bovine spongiform encephalopathy, prions, immunology, scrapie, B and T lymphocytes, cattle, cell cultures, disease prevention and control, mice.

78. Hernandez-Sanchez, Jules; Waddington, Dave; Wiener, Pamela; Haley, Chris S; Williams, John L. Genome-wide search for markers associated with bovine spongiform encephalopathy. Mammalian Genome. 2002 Mar; 13(3): 164-8 ISSN: 0938-8990.
    Abstract: A genome-wide search for markers associated with BSE incidence was performed by using Transmission-Disequilibrium Tests (TDTs). Significant segregation distortion, i.e., unequal transmission probabilities of alleles within a locus, was found for three marker loci on Chromosomes (Chrs) 5, 10, and 20. Although TDTs are robust to false associations owing to hidden population substructures, it cannot distinguish segregation distortion caused by a true association between a marker and bovine spongiform encephalopathy (BSE) from a population-wide distortion. An interaction test and a segregation distortion analysis in half-sib controls were used to disentangle these two alternative hypotheses. None of the markers showed any significant interaction between allele transmission rates and disease status, and only the marker on Chr 10 showed a significant segregation distortion in control individuals. Nevertheless, the control group may have been a mixture of resistant and susceptible but unchallenged individuals. When new genotypes were generated in the vicinity of these three markers, evidence for an association with BSE was confirmed for the locus on Chr 5.
    NAL call no. QL738.5.M359
    Descriptors: BSE, cattle, Transmission-Disequilibrium Tests, Chromosomes 5, 10, 20, genome-wide markers

79. Herrmann, Lynn M.; Baszler, Timothy V.; Knowles, Donald P.; Cheevers, William P. PrPSc is not detected in peripheral blood leukocytes of scrapie-infected sheep: Determining the limit of sensitivity by immunohistochemistry. Clinical and Diagnostic Laboratory Immunology. March, 2002; 9 (2): 499-502.
    Descriptors: Peripheral blood leukocytes, scrapie-infected sheep, presence of PrPSc, dissociated retropharyngeal lymph node (DRLN) cells, immunohistochemistry, detection methods.

80. Hildebrandt, G.; Luy, J.; Simon, O. Kannibalismus und Rinderwahn: Ein Argument gegen jegliche Tiermehlfuetterung? [Cannibalism and mad cows: An argument against feeding of mammalian meat and bone meal (MBM)?] Tieraerztliche Umschau. 1 Februar, 2002; 57 (2): 77-89. In German.
    NAL call no. 41.8 T445
    Descriptors: bovine spongiform encephalopathy, cattle, feed formulations, mammalian meat and bone meal, cannibalism, offal recycling, rendering.

81. Hill, Andrew E.; Collinge, John Species-barrier-independent prion replication in apparently resistant species. Acta Pathologica, Microbiologica, et Immunologica Scandinavica. 2002 Jan; 110(1): 44-53. ISSN: 0903-4641.
    Abstract: Prion diseases of humans and animals are associated with the accumulation of an abnormal isoform (PrP(Sc)) of the host-encoded prion protein (PrP(C)). Transmission of these diseases between mammalian species is usually limited by a 'species barrier', which can be mediated by differences in primary sequence of the prion protein between donor and host species. Studies on species barriers usually rely on the development of clinical disease in inoculated animals as an assessment of susceptibility in a particular host. Recent studies by a number of groups have demonstrated that the absence of clinical symptoms does not necessarily exclude transmission of prion disease across a species barrier. Such results indicate that subclinical or carrier states exist in these diseases, which has public health implications regarding human exposure to BSE prions and iatrogenic transmission from apparently healthy humans. Here the issue of subclinical prion diseases is reviewed and implications are discussed.
    NAL call no. QR1.A6
    Descriptors: prion diseases, hamsters, mice, laboratory animal models, subclinical and carrier state implications for human disease, BSE, iatrogenic transmission, review.

82. Holada, Karel; Vostal, Jaroslav G.; Theisen, Patrick W.; MacAuley, Claudia; Gregori, Luisa; Rohwer, Robert G. Scrapie infectivity in hamster blood is not associated with platelets. Journal of Virology. May, 2002; 76 (9): 4649-4650. ISSN: 0022-538X.
    DOI: 10.1128/JVI.76.9.4649-4650.2002
    Abstract: The infectivity of hamster scrapie strain 263K was measured in platelets isolated from blood pooled from six hamsters with clinical scrapie. The total number of infectious doses present in the blood pool was 220, out of which only 3.5 infectious doses were associated with platelets. A larger proportion of the total infectivity was recovered from the mononuclear leukocyte fraction. This result indicates that platelets are not the source of blood-borne infectivity in transmissible spongiform encephalopathy-infected hamsters.
    NAL call no. QR360.J6
    Descriptors: scrapie strain 263K, infected hamster blood, platelet fraction analysed, mononuclear leukocytes, infective source, scrapie transmission.

83. Hooper, Nigel M. Prion disease: Close encounters of the cellular kind. Current Biology. April 2, 2002; 12 (7): R248-R249.
    DOI: 10.1016/S0960-9822(02)00783-2
    NAL call no. QH301.C85
    Descriptors: prion diseases, BSE, CJD.

84. Horby, Peter. Rendering beef safe. Clinical Infectious Diseases. 1 January, 2002; 34 (1): 129. ISSN: 1058-4838.
    NAL call no. RC111 R4
    Descriptors: food products, beef, human health risks, BSE.

85. Hsich, Gary; Kenney, Kimbra; Gibbs, Clarence J.Jr; Harrington, Michael, G. Method of detecting transmissible spongiform encephalopathies. Official Gazette of the United States Patent and Trademark Office Patents. [e-file] June 18, 2002; 1259 (3): No Pagination.
    Journal URL: www.uspto.gov/web/menu/patdata.html
    NAL call no. T223 A22
    Descriptors: detection method, 14-3-3 proteins, cerebrospinal fluid, elevated levels indicate transmissible spongiform encephalopathies, CJD in humans, BSE in cattle.

86. Huang, Fang Ping; Farquhar, Christine F.; Mabbott, Neil A.; Bruce, Moira E.; MacPherson, G. Gordon. Migrating intestinal dendritic cells transport PrP(Sc) from the gut. Journal of General Virology. 2002 Jan; 83(Pt 1): 267-71. ISSN: 0022-1317.
    Abstract: Bovine spongiform encephalopathy, variant Creutzfeldt-Jakob disease (vCJD) and possibly also sheep scrapie are orally acquired transmissible spongiform encephalopathies (TSEs). TSE agents usually replicate in lymphoid tissues before they spread into the central nervous system. In mouse TSE models PrP(c)-expressing follicular dendritic cells (FDCs) resident in lymphoid germinal centres are essential for replication, and in their absence neuroinvasion is impaired. Disease-associated forms of PrP (PrP(Sc)), a biochemical marker for TSE infection, also accumulate on FDCs in the lymphoid tissues of patients with vCJD and sheep with natural scrapie. TSE transport mechanisms between gut lumen and germinal centres are unknown. Migratory bone marrow-derived dendritic cells (DCs), entering the intestinal wall from blood, sample antigens from the gut lumen and carry them to mesenteric lymph nodes. Here we show that DCs acquire PrP(Sc) in vitro, and transport intestinally administered PrP(Sc) directly into lymphoid tissues in vivo. These studies suggest that DCs are a cellular bridge between the gut lumen and the lymphoid TSE replicative machinery.
    NAL call no. QR360.A1J6
    Descriptors: bovine spongiform encephalopathy, NvCreutzfeldt-Jakob disease, vCJD, sheep scrapie, orally acquired transmissible spongiform encephalopathies, TSEs, mouse models, transport of dendritic cells directlyinto lymphoid tissues in vivo, replicatation.

87. Ingrosso, Loredana; Vetrugno, Vito; Cardone, Franco; Pocchiari, Maurizio Molecular diagnostics of transmissible spongiform encephalopathies. Trends in Molecular Medicine. June, 2002; 8 (6): 273-280.
    Descriptors: post mortem diagnosis, immunochemical based kits, PrPSc, preclinical screening tests, blood, blood derived products, meat safety, Europe.

88. Ironside, James W. Neuropathology of variant Creutzfeldt-Jakob disease. [Neuropathologie de la maladie variant de Creutzfeldt Jakob]. Comptes Rendus Biologies. Janvier, 2002; 325 (1): 27-31.
    DOI: 10.1016/S1631-0691(02)01381-1
    NAL call no. Q2 C6
    Descriptors: human prion diseases, NvCreutzfeldt-Jakob Disease, BSE, florid cluster plaques, cerebrum, cerebellum, PrPRES, morphology, histology, biochemistry, codon 129, compared to CJD.

89. Ironside, J.W.; McCardle, L.; Horsburgh, A.; Lim, Z.; Head, M.W. Pathological diagnosis of variant Creutzfeldt-Jakob disease. APMIS. Acta Pathologica, Microbiologica, et Immunologica Scandinavica 2002 Jan; 110(1): 79-87 ISSN: 0903-4641.
    Abstract: The neuropathological and biochemical features of the 89 histologically confirmed cases of variant Creutzfeldt-Jakob disease (vCJD) diagnosed up to the end of October 2001 in the UK are reviewed. Histology of the central nervous system, lymphoid tissues and other organs was accompanied by immunocytochemistry and Western blot analysis of the disease-associated form of the prion protein (PrP(RES)). All patients with vCJD were methionine homozygotes at codon 129 of the PrP gene. The pathology of vCJD showed relatively uniform morphological and immunocytochemical characteristics, which were distinct from other forms of CJD. PrP(RES) accumulation was widespread in lymphoid tissues in vCJD, but was not identified in other non-neural tissues. PrP(RES) in vCJD brain tissue showed a uniform glycotype pattern distinct from sporadic CJD. Given the increasingly widespread occurrence of bovine spongiform encephalopathy in Europe and Asia, there is a major need for widespread CJD surveillance. This should be accompanied by a multidisciplinary laboratory approach to the investigation and diagnosis of all forms of CJD, with the need to investigate autopsy tissues from suspected cases by the histological and biochemical techniques described herein.
    NAL call no. QR1.A6
    Descriptors: NvCreutzfeldt-Jakob Disease, histology, histology, immunocytochemistry, Western blot analysis, CNS, lymphoid tissue, other organs.

90. Kaaden, O.R.; Eichhorn, W.; Essbauer, S. Recent developments in the epidemiology of virus diseases. J Vet Med, Ser B. Berlin : Blackwell Wissenschafts-Verlag. Feb 2002. v. 49 (1) p. 3-6. ISSN: 0931-1793.
    Abstract: There is continual variation in viral epidemics regarding clinical symptoms, duration and disappearance, and the emergence of new diseases. This can be observed in both human and animal diseases. This evolution of virus diseases is mainly related to three factors: aetiological agent, host and environment. As far as genetic alterations of the virus are concerned, two major mechanisms are involved: mutations such as recombination and reassortment; and selection for resistance or susceptibility. This review focuses on the epidemiology of newly emerged virus diseases in man and animals, such as acquired immunodeficiency syndrome, haemorraghic fevers, bovine spongiform encephalopathy, canine haemorraghic disease and respiratory syndrome in horses.
    NAL call no. 41.8 Z52
    Descriptors: human, livestock, viral diseases, epidemiology, epidemics, symptoms, duration, evolution, etiology, genetic variation, mutations, recombination, natural selection, hemorrhagic fevers, bovinespongiform encephalopathy, literature reviews, acquired immune deficiency syndrome, canine hemorrhagic disease, respiratory syndrome in horses.

91. Kahler, Susan C. The rationale for ridding U.S. of scrapie. Journal of the American Veterinary Medical Association. 2002 May 1; 220(9): 1280-1. ISSN: 0003-1488
    NAL call no. 41.8 AM3
    Descriptors: BSE, bovine spongiform encephalopathy, prevention and control, scrapie, cattle, sheep, animal feed formations and processing, contaminated feeds, prion diseases, US.

92. Kao, R. R.; Gravenor M.B.; Baylis, M.; Bostock, C.J.; Chihota, C. M.; Evans, J.C.; Goldmann, W.; Smith, A. J. A.; McLean, A.R. The potential size and duration of an epidemic of bovine spongiform encephalopathy in British sheep. Science. Washington, DC. 11 January, 2002; 295 (5553): 332-335. ISSN: 0036-8075
    Abstract: Because there is a theoretical possibility that the British national sheep flock is infected with bovine spongiform encephalopathy (BSE), we examined the extent of a putative epidemic. An age cohort analysis based on numbers of infected cattle, dose responses of cattle and sheep to BSE, levels of exposure to infected feed, and number of BSE-susceptible sheep in the United Kingdom showed that at the putative epidemic peak in 1990, the number of cases of BSE-infected sheep would have ranged from fewer than 10 to about 1500. The model predicts that fewer than 20 clinical cases of BSE in sheep would be expected in 2001 if maternal transmission occurred at a rate of 10%. Although there are large uncertainties in the parameter estimates, all indications are that current prevalence is low; however, a simple model of flock-to-flock BSE transmission shows that horizontal transmission, if it has occurred, could eventually cause a large epidemic.
    NAL call no. 470 Sci2
    Descriptors: BSE, bovine spongiform encephalopathy, cattle, sheep diseases, horizontal disease transmission, cohort studies, genetic predisposition to Disease, Great-Britain, epidemiology, logistic models, prevalence, prions chemistry and genetics, probability, scrapie epidemiology and transmission, sheep genetics.

93. Kaup F. J.; Schwibbe, M. Primaten als Versuchstiere. [Nonhuman primates as laboratory animals.] DTW Deutsche-Tieraerztliche-Wochenschrift. Marz, 2002; 109 (3): 104-108. In German.
    NAL call no. 41.8 D482
    Descriptors: non-human primates, biomedical models, disease models, viral infections, transmissible spongiform encephalopathies.

94. Kersseboom, R.; Koekoek, S. C.; Richardus, J. H. Het risico van de variant van de ziekte van Creutzfeldt-Jakob in Nederland en het effect van preventieve maatregelen. [The risk of variant Creutzfeldt-Jakob disease in the Netherlands and the effect of preventive measures]. Nederlands Tijdschrift voor Geneeskunde. 2002 Apr 20; 146(16): 754-9 ISSN: 0028-2162. In Dutch.
    Abstract: Variant Creutzfeldt-Jakob disease (vCJD) is a fatal and untreatable neurological disease, in which pathogenic prions (PrPSc) are involved. There is convincing epidemiological and experimental evidence that vCJD is a human expression of bovine spongiform encephalopathy (BSE). The risk of transmission of pathogenic prions which cause vCJD to humans is influenced by the species barrier, genetic susceptibility of the host, dose of infection and route of exposure. Transmission of pathogenic prions from bovines to humans is possible through meat products containing nerve and lymphatic tissue, and through medical products derived from bovine material. Human to human transmission is, in principle, also possible via blood and blood-derived products, human organs and tissues for transplantation, and through surgical instruments. Preventive measures to reduce transmission from bovines to humans have been introduced step by step in the Netherlands since 1989. With proper implementation, the current risk of becoming infected by Dutch meat products is small. It is very likely, however, that in the past decade BSE-infected bovines have entered the food chain. The Dutch population has also been exposed to foreign infected meat products. Measures to prevent human to human transmission are currently being improved in the Netherlands. It is expected that cases of vCJD will occur in the Netherlands in the future, but the number cannot be estimated accurately. The presence of PrPSc in both the livestock and in the human population in the Netherlands constitutes a permanent public health threat and is a reason for continued vigilance and active prevention.
    Descriptors: NvCJD, transmission of pathogenic prions, control measures, public health concerns, Netherlands, risk assessment, level of PrPSc in livestock and people.

95. Kirk, Sara F. L.; Greenwood, Darren; Cade, Janet E.; Pearman, Alan D. Public perception of a range of potential food risks in the United Kingdom. Appetite. June, 2002; 38 (3): 189-197. ISSN: 0195-6663
    DOI: 10.1006/appe.2001.0478
    NAL call no. QP141.A1A64
    Descriptors: public/consumer survey, potential food risks, BSE, bovine spongiform encephalopathy, Salmonella, 1998, 1999, UK.

96. Kneipp, Janina; Beekes, Michael; Lasch, Peter; Naumann, Dieter. Molecular changes of preclinical scrapie can be detected by infrared spectroscopy. Journal of Neuroscience. April 15, 2002; 22 (8): 2989-2997.
    URL: http://www.jneurosci.org/cgi/content/abstract/22/8/2989
    Descriptors: infrared microspectroscopy, molecular changes, cryosections, scrapie-infected brain tissue, 263K scrapie infected hamster nervous tissue, medulla oblongata.

97. Krebs, John R.; May, Robert M.; Stumpf, Michael P.H. Theoretical models of sheep BSE reveal possibilities. Nature. 2002 Jan 10; 415(6868): 115 ISSN: 0028-0836.
    DOI: 10.1038/415115a
    NAL call no. 472 N21
    Descriptors: bovine spongiform encephalopathy, etiology, sheep diseases, animal feed formulations, cattle, disease prevention and control, public policy, risk assessment.

98. Kohnlein, C.; Poser, S. Schwerpunktheft Creutzfeldt-Jakob-Krankheit/BSE. [Creutzfeldt-Jakob disease/BSE special issue]. Deutsche Medizinische Wochenschrift. 2002 Jun 14; 127(24): 1344. ISSN: 0012-0472. In German.
    NAL call no. 448.8 D48
    Descriptors: BSE, humans, cattle, epidemiology, historical notes, risk factors, transmission, UK, Scotland.

99. Larski, Zdzislaw Niektore nowe dane dotyczace wirusologii i zakaznych gabczastych encefalopatii. [Some new data concerning virology and transmittable spongiform encephalopathies.] Medycyna-Weterynaryjna. 2002; 58 (1): 13-17. In Polish.
    NAL call no. 41.8 M463
    Descriptors: review, preclinical diagnosis, transmissible spongiform encephalopathies, TSE.

100. Laude, Hubert; Vilette, Didier; Le Dur, Annick; Archer, Fabienne; Soulier, Solange; Besnard, Nathalie; Essalmani, Rachid; Vilotte, Jean-Luc New in vivo and ex vivo models for the experimental study of sheep scrapie: Development and perspectives. Comptes Rendus Biologies. Janvier, 2002; 325 (1): 49-57.
     DOI: 10.1016/S1631-0691(02)01393-8
     NAL call no. Q2 C6
     Descriptors: sheep, scrapie, transmissible spongiform encephalopathy, TSE, transgenic mouse models-tgOv, invitro cell lines expressing ovine prion proteins, rabbit epithelial cell line.

101. Lawlor, D.; Hockley, J.; Hawkins, S.A.C.; Simmons, M.M.; Matthews, D. The TSE Archive at VLA Weybridge. Research in Veterinary Science. April, 2002; 72 (Supplement A): 46. 56th Annual Conference of the Association of Veterinary Teachers and Research Workers on Current Topics in Veterinary Science, Scarborough, England, UK, March 25-27, 2002
     NAL call no. 41.8 R312
     Descriptors: transmissible spongiform encephalopathies, prions.

102. Lehmann, Sylvain. Metal ions and prion diseases. Current Opinion in Chemical Biology. April, 2002; 6 (2): 187-192.
     Descriptors: prion, metal ions, BSE, other prion diseases.

103. Lehmann, Sylvain; Beranger, Florence; Solassol, Jerome; Ceschia, Audrey; Perrier, Veronique; De Gassart, Aude; Vilette, Didier; Laude, Hubert; Kellermann, Odile; Mange, Alain. Modeles en culture cellulaire des encephalopathies spongiformes transmissibles. [Cell culture models of transmissible spongiform encephalopathies.] Comptes Rendus Biologies. Janvier, 2002; 325 (1): 59-65.
     DOI: 10.1016/S1631-0691(02)01391-4
     NAL call no. Q2 C6
     Descriptors: invitro cell cultures, experimental models, prion infected lines, wild-type, mutated or chimeric prion proteins, useful for investigating the biology of pathogenicity.

104. Levieux, A.; Rivera, V.; Levieux, D. Immunochemical control of the species origin of porcine crude heparin and detection of ovine and caprine materials. Journal of Pharmaceutical and Biomedical Analysis. 2002 Jan 1. 27(1-2): 305-313 ISSN: 0731-7085
     Abstract: As a consequence of the outbreak of bovine spongiform encephalopathy (BSE), ruminants materials have been generally banned from the production of heparin. Immunochemical methods have been recently developed for the control of the raw materials used by manufacturers of materials such as porcine mucosa and for the detection of bovine crude heparins. To certify the porcine origin of crude porcine heparins and to exclude ovine or caprine materials, new ELISAs were developed. Rabbit antisera were produced against species-specific antigenic contaminants present in crude heparins or in eluted materials (EM) from the chromatographic step of the purification process. When analysed by line immunoelectrophoresis, these antisera revealed five to eleven antigenic contaminants in the EMs, the major one being the most anodic and predominant antigen in crude heparins. Using the best antisera, competitive indirect ELISAs were optimised. They allowed the detection of porcine, ovine and caprine crude heparins down to a dilution of 0.6 to 1.5 parts per 1000, with CVs ranging from 3 to 12%. These ELISAs complete the set of immunological techniques which can be routinely used by heparin manufacturers to secure their supply chain.
     Descriptors: BSE, bovine based pharmaceuticals, health risks, ELISA test, antibodies.

105. Lucker, Ernst; Hardt, Michael; Groschup, Martin H. Detection of CNS and PrPSc in meat products. Berliner und Munchener Tierarztliche Wochenschrift 2002 Mar-Apr; 115(3-4): 111-7. ISSN: 0005-9366.
     Abstract: Several methods for the detection of tissues of the central nervous system (CNS) in meat products have been developed and partly validated for use in official food control as pertaining to human BSE-exposure risk. So far, however, methods for the detection of abnormal prion protein (PrPSc) were not evaluated for their potential applicability to the matrix of heat treated meat products. We developed a micro technological procedure for the preparation of meat products suitable for high security laboratories as masses were 6 to 8 orders of magnitude lower than in conventional meat technology. Thus it was possible to produce standard micro sausages containing defined amounts of bovine BSE-positive brain. This material showed all characteristics of normal meat products and a homogeneous distribution of brain as indicated by NSE and GFAP western immunoblotting and GFAP immunometric analyses. Using a commercially available and certified immunometric assay for detection of PrPSc in untreated brain it was possible to detect BSE-positive CNS down to a content of 0.25% in heat treated meat products. We found a high correlation between PrPSc OD-values and CNS content and linearity up to 10% CNS. In 30 samples of retail meat products no sample transgressed the official cut off value for untreated bovine brain. Further studies are needed to show whether an increase of sensitivity in PrPSc detection from the meat product matrix is possible, in particular by optimisation of the extraction procedure.
     NAL call no. 41.8 B45
     Descriptors: CNS, PrPSc, detection of central nervous tissue in meat, immunometric assay, detection of PrPSc in untreated brain.

106. Lucker, Ernst; Schlottermuller, Beate; Martin, Antje Studies on contamination of beef with tissues of the central nervous system (CNS) as pertaining to slaughtering technology and human BSE-exposure risk. Berliner und Munchener Tierarztliche Wochenschrift. 2002 Mar-Apr; 115(3-4): 118-21 ISSN: 0005-9366
     Abstract: Contamination of beef by tissues of the central nervous system (CNS) due to slaughter technology causes some concern considering the potential health hazard by food borne exposure to the infectious agent of BSE. The present study was designed to quantify the extent of CNS contamination as pertaining to stunning and splitting technology. Of the 726 animals 48 contained a total of 58 emboli-like particles in lungs and/or right ventricles. The incidence of emboli-like particles was found to be slightly higher in animals slaughtered without pithing (5.9%) than in the animals slaughtered with pithing (4.1%). Of the 58 emboli-like particles only two were positive in the anti-NSE western immunoblotting (0.3% of the 726 animals). The immuno reaction of these NSE-positive particles was several orders of magnitude lower as obtained by pure brain material. The microscopical analysis of the two NSE-positive emboli-like particles for presence of CNS-like tissues was negative. Following splitting of carcasses by sawing with and without prior removing the spinal cord we found NSE-positive reactions in 32% and 17% of the samples, respectively. The immuno reaction, however, was predominantly comparable to standard material containing less than 0.5% CNS. Overall the results show that CNS contamination of bovine carcasses cannot be excluded by current slaughter technology. However, the additional human BSE-exposure risk can be judged to be at least minor when considering extent of contamination, dilution effects and BSE-testing.
     NAL call no. 41.8 B45
     Descriptors: cattle, slaughter processes, level of beef meat contamination, central nervous system tissue, emboli-like particles.

107. Lunney, Joan K.; Fossum, Caroline; Alm, Gunnar V.; Steinbach, Falko; Wattrang, Eva. Veterinary immunology: Opportunities and challenges. Trends in Immunology. January, 2002; 23 (1): 4-6. 6th International Veterinary Immunology Symposium, Uppsala, Sweden, July 15-20, 2001. ISSN: 1471-4906
     NAL call no. QR180 I56
     Descriptors: Veterinary medicine, animal immunology.

108. Lupi, Omar. Prions in dermatology. Journal of the American Academy of Dermatology. 2002 May; 46(5): 790-3 ISSN: 0190-9622
     Abstract: Prion diseases are uncommon fatal neurodegenerative disorders that have gained scientific importance as a result of the emergence of new forms of these diseases in both animals and humans. Prions appear to be composed principally or entirely of abnormal isoforms of a host-encoded glycoprotein. There is substantial scientific evidence to support the notion that bovine spongiform encephalopathy ("mad cow disease") has affected humans. Recent studies have demonstrated that prions can adhere easily to metal surfaces, and normal sterilization procedures are not likely to completely inactivate them. Iatrogenic transmission of prion diseases, such as Creutzfeldt-Jakob disease, was recognized after corneal transplantations, dura mater grafts, neurosurgical procedures, and the use of human hormones (growth hormone and gonadotropin). Although bovine collagen has long been recognized as a safe and biocompatible material, dermatologists should be aware of the theoretical potential for prion transmission when materials from bovine origin and products obtained from cultured cells fed with fetal or newborn calf serum are used.
     Descriptors: spongiform encephalopathies, prions, iatrogenic transmission, bovine based products, fetal or newborn calf serum, risks.

109. Mabbott, Neil A; McGovern, Gillian; Jeffrey, Martin; Bruce, Moira E. Temporary blockade of the tumor necrosis factor receptor signaling pathway impedes the spread of scrapie to the brain. Journal of Virology 2002 May; 76(10): 5131-9. ISSN: 0022-538X
     DOI: 10.1128/JVI.76.10.5131-5139.2002
     Abstract: Although the transmissible spongiform encephalopathies (TSEs) are neurodegenerative diseases, their agents usually replicate and accumulate in lymphoid tissues long before infection spreads to the central nervous system (CNS). Studies of a mouse scrapie model have shown that mature follicular dendritic cells (FDCs), which express the host prion protein (PrP(c)), are critical for replication of infection in lymphoid tissues. In the absence of mature FDCs, the spread of infection to the CNS is significantly impaired. Tumor necrosis factor alpha (TNF-alpha) secretion by lymphocytes is important for maintaining FDC networks, and signaling is mediated through TNF receptor 1 (TNFR-1) expressed on FDCs and/or their precursors. A treatment that blocks TNFR signaling leads to the temporary dedifferentiation of mature FDCs, raising the hypothesis that a similar treatment would significantly delay the peripheral pathogenesis of scrapie. Here, specific neutralization of the TNFR signaling pathway was achieved through treatment with a fusion protein consisting of two soluble human TNFR (huTNFR) (p80) domains linked to the Fc portion of human immunoglobulin G1 (huTNFR:Fc). A single treatment of mice with huTNFR:Fc before or shortly after intraperitoneal injection with the ME7 scrapie strain significantly delayed the onset of disease in the CNS and reduced the early accumulation of disease-specific PrP in the spleen. These effects coincided with a temporary dedifferentiation of mature FDCs within 5 days of huTNFR:Fc treatment. We conclude that treatments that specifically inhibit the TNFR signaling pathway may present an opportunity for early intervention in peripherally transmitted TSEs.
     NAL call no. QR360.J6
     Descriptors: scrapie, TSE's mouse scrapie model, follicular dendritic cells, replication of i nfection in lymphoid tissues, blocking tumor necrosis factor alpha, pathogenesis delays.

110. MacDiarmid, S.C. Bovine spongiform encephalopathy (BSE) in sheep? Australian Veterinary Journal 2002 Mar; 80(3): 148-9 ISSN: 0005-0423
     NAL call no. 41.8 Au72
     Descriptors: BSE, epidemiology, disease transmission, cattle, goats, sheep, population surveillance methods, risk assessment, etiology, Australia, New Zealand.

111. Marella, Mathieu; Lehmann, Sylvain; Grassi, Jacques; Chabry, Joelle. Filipin prevents pathological prion protein accumulation by reducing endocytosis and inducing cellular PrP release. Journal of Biological Chemistry, 2002 Jul 12; 277(28): 25457-64. ISSN: 0021-9258
     Abstract: Conversion of the normal membrane-bound prion protein (PrP-sen) to its pathological isoform (PrP-res) is a key event in the pathogenesis of transmissible spongiform encephalopathies. Although the subcellular sites of conversion are poorly characterized, several lines of evidence have suggested the involvement of membrane lipid rafts in the conversion process. Here we report that copper stimulates the endocytosis of PrP-sen via a caveolin-dependent pathway in both microglia and neuroblastoma cells. We show that the polyene antibiotic filipin both limits endocytosis of PrP-sen and dramatically reduces the amount of membrane-bound PrP-sen. This reduction results from a rapid and massive release of full matured PrP-sen into the culture medium. Finally, we demonstrate that filipin is a potent inhibitor of PrP-res formation into chronically infected neuroblastoma cells. Our results reinforce the role of rafts in PrP trafficking and raise the possibility that the release of PrP-sen from the plasma membrane decreases the amount of available substrate PrP-sen at the conversion sites.
     NAL call no. 381 J824
     Descriptors: cell culture, microglia, neuroblastoma cells, copper, endocytosis, Prp-sen, effects of filipin antibiotic, inhibition of PrP-res formation, nystatin pharmacology.

112. Martin, S.; Gonzalez, L.; Jeffrey, M.; Bellworthy, S.J. Differential diagnosis of BSE agent and scrapie infection of sheep. Research in Veterinary Science. April, 2002; 72 (Supplement A): 44. 56th Annual Conference of the Association of Veterinary Teachers and Research Workers on Current Topics in Veterinary Science, Scarborough, England, UK, March 25-27, 2002
     NAL call no. 41.8 R312
     Descriptors: bovine spongiform encephalopathy, scrapie, diagnostic techniques.

113. Martinsen, T.C.; Taylor, D.M.; Johnsen, R.; Waldum, H.L. Gastric acidity protects mice against prion infection? Scandinavian Journal of Gastroenterology. May, 2002; 37 (5): 497-500.
     URL: http://www.ingentaconnect.com/content/apl/sgas/2002/00000037/00000005/art00001
     NAL call no. RC799 S33
     Descriptors: transmissible degenerative encephalopathies, sheep scrapie, bovine spongiform encephalopathy, Creutzfeldt-Jakob Disease, gastric acidity and susceptibility to infection, mouse model, experimentalinfection, implications for eating contaminated meats.

114. Matorras, Roberto; Rodriguez Escudero, Francisco, J. The use of urinary gonadotrophins should be discouraged. Human Reproduction. Oxford. July, 2002; 17 (7): 1675.
     NAL call no. QP251 H85
     Descriptors: prion proteins, infectivity concerns, transmissible spongiform encephalopathies, FSH, LH.

115. McCormack, James E.; Baybutt, Herbert N.; Everington, Dawn; Will, Robert G.; Ironside, James W.; Manson, Jean C. PRNP contains both intronic and upstream regulatory regions that may influence susceptibility to Creutzfeldt-Jakob Disease. Gene. 2002 Apr 17; 288(1-2): 139-46 ISSN: 0378-1119
     Abstract: The Prion protein (PrP) plays a central role in Creutzfeldt-Jakob Disease (CJD) and other transmissible spongiform encephalopathies (TSEs). Mutations in the protein coding region of the human PrP gene (PRNP), which have been proposed to alter the stability of the PrP protein, have been linked to a number of forms of TSE. However, the majority of CJD cases are not associated with mutations in the PRNP coding region and alternative mechanisms must therefore underlie susceptibility to these forms of CJD. Transgenic mice, that over- or under-express PrP genes, have shown a correlation between the level of PrP gene expression and the incubation time of disease. Polymorphisms that lead to alterations in human PRNP gene expression, could therefore be candidates for influencing susceptibility of an individual to CJD. In order to investigate this hypothesis, we have defined an upstream and intronic regulatory region of the PRNP gene. Sequencing of these regions in controls, sporadic CJD (sCJD) and variant CJD (vCJD) patients has identified three polymorphisms, all of which are more common in sCJD patients than controls. Our data suggests that polymorphisms in the regulatory region of the PRNP gene may be a risk factor for CJD.
     NAL call no. QH442.A1G4
     Descriptors: risk factors for disease, humans, polymorphisms, regulatory regions.

116. Milhavet, Ollivier; Lehmann, Sylvain. Oxidative stress and the prion protein in transmissible spongiform encephalopathies. Brain Research. Brain Research Reviews. 2002 Feb; 38(3): 328-39 ISSN: 0165-0173
     Abstract: Transmissible spongiform encephalopathies form a group of fatal neurodegenerative disorders that have the unique property of being infectious, sporadic or genetic in origin. These diseases are believed to be the consequence of the conformational conversion of the prion protein into an abnormal isoform. Their exact pathogenic mechanism remains uncertain, but it is believed that oxidative stress plays a central role. In this article, we will first review in detail the data supporting the latter hypothesis. Subsequently, we will discuss the relationship between the prion protein and the cellular response to oxidative stress, attempting ultimately to link PrP function and neurodegeneration in these disorders.
     Descriptors: TSE, prion protein conformation, oxidative stress, pathogenic mechanism.

117. Morgenthaler, Jean Jacques; Maring, Jacques Andre; Rentsch,Markus. Method for the removal of causative agent(s) of transmissible spongiform encephalopathies from protein solutions. Official Gazette of the United States Patent and Trademark Office Patents. [e-file] June 18, 2002; 1259 (3): No Pagination
     Journal URL: www.uspto.gov/web/menu/patdata.html
     NAL call no. T223 A22
     Descriptors: non-conventional transmissible agents, causative agents, transmissible spongiform encephalopathies, contaminated protein solution, absorbent, kieselguhr, diatomaceous earth, silicic acid, clay minerals, metal hydroxide, metal oxihydrate, cellulose, perlite, bentonite, and water-insoluble synthetic polymers.

118. Newgard, Jason R.; Rouse, Glenda C.; McVicker, Jerry K. Novel method for detecting bovine immunoglobulin G in dried porcine plasma as an indicator of bovine plasma contamination. Journal of Agricultural and Food Chemistry 2002 May 22; 50(11): 3094-7. ISSN: 0021-8561
     Abstract: Current U.S. Food and Drug Administration regulationsprohibit feeding of protein derived from mammalian tissue, excluding blood and blood products and any product that consists entirely of porcine or equine protein. A novel lateral flow immunoassay device has been developed that can quickly and qualitatively determine the presence of bovine immunoglobulin G (IgG), a major component in blood products, at very low levels (0.01% v/v). The device can be used to test for bovine IgG commingling in spray-dried porcine plasma used in the feed industry. Producers and consumers alike could use this device to verify product content at threshold levels.
     NAL call no. 381 J8223
     Descriptors: US FDA, animal feeds, and swine or horse protein comtaminants, immunoassay for bovine IgG contaminants, BSE control and prevention.

119. Nair, Bindu; Elmore, Amy R. Final report on the safety assessment of Human Placental Protein, Hydrolyzed Human Placental Protein, Human Placental Enzymes, Human Placental Lipids, Human Umbilical Extract, Placental Protein, Hydrolyzed Placental Protein, Placental Enzymes, Placental Lipids, and Umbilical Extract. International Journal of Toxicology. 2002; 21 (Supplement 1): 81-91.
     NAL call no. RA1190 J61
     Descriptors: cosmetic ingredients, human and animal pracental proteins, transmissible spongiform encephalopathies risks, EU prohibited use, FDA, toxicology recommendations.

120. Narang, Harash. A critical review of the nature of the spongiform encephalopathy agent: protein theory versus virus theory. Experimental Biology and Medicine. 2002 Jan; 227(1): 4-19. ISSN: 1535-3702.
     Abstract: All spongiform encephalopathies (SEs) result in brain disorders brought about by a slow virus. Since the origin of bovine SE (BSE), the infectious nature of the disease has been firmly established. Tubulofilamentous particles/scrapie termed nemavirus (NVP) and scrapie-associated fibrils (SAF) are ultrastructural markers, whereas protease-resistant protein (PrP(sc)) is a protein marker. The PrP molecules aggregate to form SAF. Each NVP consists of three layers: an outer protein coat, an intermediate ssDNA layer, and inner PrP/SAF. Therefore, ssDNA and PrP/SAF are physically associated with each other. The existence of at least 20 stable strains of SEs implies that a nucleic acid molecule serves as the information molecule. Animals inoculated with PrP(sc) do not develop the clinical disease, however, ssDNA purified from scrapie-hamster brains by alkaline gel electrophoresis mixed with binding proteins before inoculation developed the clinical disease. It appears that an "accessory protein" coded by the ssDNA of the NVP interacts with normal PrP(c) molecules, resulting in their conversion to PrP(sc)/SAF. The pathogenesis process in the infected animal, with increasing incubation periods, reveals that larger amounts of normal PrP molecules are modified to form SAF. This interferes with the normal supply of PrP to cell membranes, which become disrupted and eventually fragment, resulting in the vacuoles typical of those found in the SEs. Critical review of scientific literature has demonstrated that the agent contains a DNA genome.
     NAL call no. QH301 E9
     Descriptors: bovine spongiform encephalopathy, brain disorders, scrapie, PrPSc, interaction between nemavirus and scrapie associated fibrils cause the disease, pathogenesis.

121. Nunnally, Brian K. It's a mad, mad, mad, mad cow: A review of analytical methodology for detecting BSE/TSE. Trends in Analytical Chemistry. February, 2002; 21 (2): 82-89.
     DOI: 10.1016/S0165-9936(01)00134-0
     NAL call no. QD71T7
     Descriptors: review, analytical detection, bovine spongioform encephalopathy, BSE, transmissible spongioform encephalopathy, TSE, golden Syrian hamster bioassay, immunoassays and capillary electrophoresis methods, blood, bio-fluids.

122. Ockerman, H. W.; Basu, L. Update on Bovine Spongiform Encephalopathy (BSE, mad cow disease). Special Circular Ohio Agricultural-Research and Development Center. 2002, No.183, 61-65; 5 ref.
     NAL call no. 100 Oh3S
     Descriptors: BSE, state of the U.S. cattle industry.

123. Oldstone, Michael B.A.; Race, Richard; Thomas, Diane; Lewicki,Hanna; Homann, Dirk; Smelt, Sara; Holz, Andreas; Koni, Pandelakis; Lo, David; Chesebro, Bruce; Flavell, Richard. Lymphotoxin-alpha- and lymphotoxin-beta-deficient mice differ in susceptibility to scrapie: evidence against dendritic cell involvement in neuroinvasion. Journal of Virology 2002 May; 76(9): 4357-63. ISSN: 0022-538X.
     Abstract: Transmissible spongiform encephalopathy or prion diseases are fatal neurodegenerative disorders of humans and animals often initiated by oral intake of an infectious agent. Current evidence suggests that infection occurs initially in the lymphoid tissues and subsequently in the central nervous system (CNS). The identity of infected lymphoid cells remains controversial, but recent studies point to the involvement of both follicular dendritic cells (centers is dependent on lymphotoxin alpha (LT-alpha) and LT-beta signaling components. We report here that by the oral route, LT-alpha -/- mice developed scrapie while LT-beta -/- mice did not. Furthermore, LT-alpha -/- mice had a higher incidence and shorter incubation period for developing disease following inoculation than did LT-beta -/- mice. Transplantation of lymphoid tissues from LT-beta -/- mice, which have cervical and mesenteric lymph nodes, into LT-alpha -/- mice, which do not, did not alter the incidence of CNS scrapie. In other studies, a virus that is tropic for and alters functions of CD11c(+) cells did not alter the kinetics of neuroinvasion of scrapie. Our results suggest that neither FDC nor CD11c(+) cells are essential for neuroinvasion after high doses of RML scrapie. Further, it is possible that an as yet unidentified cell found more abundantly in LT-alpha -/- than in LT-beta -/- mice may assist in the amplification of scrapie infection in the periphery and favor susceptibility to CNS disease following peripheral routes of infection. FDC) and CD11c(+) lymphoid dendritic cells. FDC generation and maintenance in general.
     NAL call no. QR360 J6
     Descriptors: scrapie, oral intake, infectious material, lymphoid tissue, NCS, LT alpha mice, LT beta mice, incubation periods.

124. Paisley, Larry G. Monitoring and analysis of bovine spongiform encephalopathy (BSE) testing in Denmark using statistical models. APMIS. January, 2002; 110 (1): 61-70.
     Abstract: The evolution of monitoring and surveillance for bovine spongiform encephalopathy (BSE) from the phase of passive surveillance that began in the United Kingdom in 1988 until the present is described. Currently, surveillance for BSE in Europe consists of mass testing of cattle slaughtered for human consumption and cattle from certain groups considered to be at higher risk of having clinical or detectable BSE. The results of the ongoing BSE testing in Denmark have been analyzed using two statistical approaches: the "classical" frequentist and the Bayesian that is widely used in quantitative risk analysis. The analyses were intended to provide information for decision-markers, the media and the public as well as to provide inputs for future BSE surveillance models. The results to date suggest that the total number of BSE cases that will be found in Denmark in 2001 will not exceed 16.
     NAL call no. QR1.A6
     Descriptors: cattle, prions, bovine spongiform encephalopathy, disease surveillance, statistical methods, epidemiology, Denmark.

125. Parnham, D.W.; Foster, J. D.; Hunter, N. Immunocytochemical tales of the unexpected and the power of the PET Blot. Research in Veterinary-Science. April, 2002; 72 (Supplement A): 47-48. 56th Annual Conference of the Association of Veterinary Teachers and Research Workers on Current Topics in Veterinary Science, Scarborough, England, UK, March 25-27, 2002
     NAL call no. 41.8 R312
     Descriptors: prion diseases, diagnostic measures.

126. Pedersen, Nils Strandberg; Smith, Else. Prion diseases: epidemiology in man. APMIS Acta Pathologica, Microbiologica, et Immunologica Scandinavica 2002 Jan; 110(1): 14-22 ISSN: 0903-4641.
     Abstract: Prion disease in man was first described as Creutzfeldt-Jacob disease (CJD) in the 1920s. CJD may have three different origins: sporadic, familial, due to mutations in the prion gene, or infectious, due to iatrogenic exposure to infectious brain material. As an example of the latter, kuru, in Papua New Guinea, was a variant of CJD transmitted by cannibalism. Between 1957 and 1982 more than 2500 died of kuru. Sporadic CJD is the most common form of CJD and occurs with an incidence of around one per million in most parts of the world. Familial CJD accounts for approximately 10% of all European cases of CJD, and is associated with inherited mutations of the prion protein gene, caused by one of the 24 single amino acid substitutions or insertions of octapeptide repeats. CJD caused by infections involves either iatrogenic cases of CJD, resulting from exposure to infectious brain, pituitary or ocular tissue, or from ingestion of infected food items. As of today, a few hundred iatrogenic cases of CJD have been diagnosed worldwide, the majority due to transmission by cadaveric pituitary HCG. So far, 111 cases of vCJD have been diagnosed caused by BSE-contaminated food. The size of the epidemic is still unclear and worst-case scenarios indicate that we may expect many thousands of cases in the future.
     NAL call no. QR1.A6
     Descriptors: Creutzfeldt-Jacob disease, CJD, sporadic, familial, kuru, NvCJD, BSE, contaminate meat products, epidemiology, cannabilism, cornal transplants, iatrogenic disease epidemiology, prion diseases, prion genetics and biochemistry, zoonotic disease.

127. Polak, Miroslaw P.; Rozek, Wojciech; Zmudzinski, Jan F. Monitoring BSE. [BSE monitoring.] Medycyna Weterynaryjna. 2002; 58 (4): 265-266. In Polish.
     NAL call no. 41.8 M463
     Descriptors: review paper, active surveillance principles, bovine spongiform encephalopathy, Switzerland, EU, Poland, diagnostic tests.

128. Polak, M.P.; Rozek, W.; Zmudzinski, J.F. Monitoring BSE w Polsce. [BSE monitoring in Poland.] Medycyna Weterynaryjna. 2002; 58 (5): 344-347. In Polish.
     NAL call no. 41.8 M463
     Descriptors: diagnostic tests, Prionics-Check(R), monitoring of BSE, EU, cattle, randon sampling.

129. Poser, Charles M. Notes on the history of the prion diseases. Part I. Clinical Neurology and Neurosurgery. 2002 Jan; 104(1): 1-9 ISSN: 0303-8467.
     Abstract: The astute observation by William Hadlow, an American veterinary neuropathologist of the similarity between the histopathology of kuru, an obscure disease of the primitive tribe in New Guinea, and scrapie of sheep, was the first clue to the etiology of the transmissible spongiform encephalopathies (TSE). The knowledge that scrapie was transmissible but only after an unusually long incubation period, that the causative agent was highly resistant to heat and formalin, and that it seemed to be able to replicate in the absence of nucleic acid, eventually led to the discovery of the prion by Stanley Pruisner and the still controversial protein-only hypothesis of etiology of the TSE.
     Descriptors: kuru, scrapie, transmissible spongiform encephalopathies, prion disease theory.

130. Poser, Charles M. Notes on the history of the prion diseases. Part II. Clinical Neurology and Neurosurgery. 2002 May; 104(2): 77-86 ISSN: 0303-8467
     Abstract: The protein-only theory of transmission of the prion diseases remains controversial. Other mechanisms such as the virus, virino, and viroid hypotheses are still under consideration. All these fit in the concept of 'slow' infections that had been proposed in 1954 by Bjorn Sigurdsson, an Icelandic pathologist. Regardless of the exact mode of infection, the presence of prions in the brain has served to unite Creutzfeldt-Jakob disease (CJD), the Gerstmann-Straussler-Scheinker syndrome and fatal familial insomnia, as well as scrapie and a number of other animal diseases, into a single pathological entity, the transmissible spongiform encephalopathies. The appearance of bovine spongiform encephalopathy in the United Kingdom and its putative relationship to new variant CJD, have put a new and unpredictable light on these unusual and uncommon diseases.
     Descriptors: disease transmission theories, BSE, Creutzfeldt-Jakob disease, CJD, the Gerstmann-Straussler-Scheinker syndrome, fatal familial insomnia, scrapie, NvCJD, pathological agent.

131. Poser, S.. BSE - Eine Ursache fur die Creutzfeldt-Jakob-Krankheit? [BSE - A cause for Creutzfeldt-Jakob disease?] Deutsche Medizinische Wochenschrift 2002 Feb 15; 127(7): 311 ISSN: 0012-0472. In German.
     NAL call no. 448.8 D48
     Descriptors: BSE, bovine spongiform encephalopathy, transmission, diagnosis, epidemiology, biological markers, cattle, iatrogenic disease, risk factors, France, Germany, Great Britain, Ireland.

132. Poser, S.; Zerr, I.; Felgenhauer, K. Die neue Variante der Creutzfeldt-Jakob-Krankheit. [New variant Creutzfeldt-Jakob disease]. Deutsche Medizinische Wochenschrift. 2002 Feb 15; 127(7): 331-4 ISSN: 0012-0472. In German.
     NAL call no. 448.8 D48
     Descriptors: NvCreutzfeldt-Jakob Disease, epidemiology, BSE, bovine spongiform encephalopathy, diagnosis, transmission, contaminated meat, human risk factors, incidence of disease.

133. Ramsay, A. R145: Evolution of a TSE diagnostic antibody. Research in Veterinary Science. April, 2002; 72 (Supplement A): 48. 56th Annual Conference of the Association of Veterinary Teachers and Research Workers on Current Topics in Veterinary Science, Scarborough, England, UK, March 25-27, 2002.
     NAL call no. 41.8 R312
     Descriptors: transmissible spongiform encephalopathies, diagnostic test, R145 antibody, development.

134. Redman, C.A.; Coen, P.G.; Matthews, L.; Lewis, R. M.; Dingwall, W.S.; Foster, J.D.; Chase-Topping, M. E.; Hunter, N.; Woolhouse, M. E. J. Comparative epidemiology of scrapie outbreaks in individual sheep flocks. Epidemiology and Infection 2002 Jun; 128(3): 513-21.
     Abstract: Data recording the course of scrapie outbreaks in 4 sheep flocks (2 in Cheviot sheep and 2 in Suffolks) are compared. For each outbreak the data on scrapie incidence and sheep demography and pedigrees cover periods of years or decades. A key finding is that the incidence of clinical cases peaks in sheep 2-3 years old, despite very different forces-of-infection. This is consistent with age-specific susceptibility of sheep to scrapie, as has been reported for cattle to bovine spongiform encephalopathy and for humans to variant Creutzfeldt-Jakob disease. Scrapie incidence was higher in ewes than rams and at certain times of years, though these effects were not consistent between flocks. There was no evidence for high levels of vertical transmission.
     NAL call no. RA651.A1E74
     Descriptors: scrapie, sheep, disease outbreaks, vertical transmission, male and female comparisons.

135. Resende, C.; Parham, S.N.; Tinsley, C.; Ferreira, P.; Duarte, J.A.B.; Tuite, M.F. The Candida albicans Sup35p protein (CaSup35p): function, prion-like behaviour and an associated polyglutamine length polymorphism. Microbiology. 2002, 148: 4. ISSN: 1049-1060.
     NAL call no. QR1 J64
     Descriptors: prions, yeast protein, comparison, biochemistry, Sup35pprotein.

136. Riley, Maria Louise; Leucht, Christoph; Gauczynski, Sabine; Hundt, Christoph; Brecelj, Martina; Dodson, Guy; Weiss, Stefan High-level expression and characterization of a glycosylated covalently linked dimer of the prion protein. Protein Engineering. June, 2002; 15 (6): 529-537.
     NAL call no. TP248 P77P763
     Descriptors: prion protein dimers, scrapie prion protein PrPSc, PrPc, human dimeric form, digestibility by proteinase, life cycle of proteins.

137. Rosted, Palle; Jorgensen, Viggo Kragh. Prion strain causing bovine spongiform encephalopathy (BSE) in cattle. Annals of Surgery. 2002 Feb; 235(2): 311. ISSN: 0003-4932.
     Descriptors: cats diseases, prion diseases, BSE, cattle.

138. Sabate, Raimon; Estelrich, Joan. Aggregation characteristics of ovalbumin in beta-sheet conformation determined by spectroscopy. Biopolymers. 2002; 67(2): 113-20 ISSN: 0006-3525.
     Abstract: Protein misfolding and aggregation are involved in a number of the so-called "conformational" diseases (e.g., transmissible spongiform encephalopathies and Alzheimer disease). The development of rational strategies to interfere with aggregation is a potential therapeutic approach that requires complete knowledge of the aggregation process. We studied the aggregation of ovalbumin in beta-sheet conformation using mainly the spectral changes in the spectra of two dyes (Congo Red and pinacyanol) caused by the aggregates. We assumed a linear model of polymerization that fit to the experimental data. The critical aggregation constant, concentration of half-aggregation, nucleation parameter, growth parameter, and number of aggregation and free energy changes (total and per residue) were determined as aggregation-related parameters. Beta-Ovalbumin aggregates in a cooperative way. Moreover, the differences between such parameters obtained with Congo Red and pinacyanol suggest that each dye interacts with the protein in its own way.
     NAL call no. 381 B524
     Descriptors: protein structure, misfolding, prions, transmissible spongiform encephalopathies.

139. Sasaki, Kensuke; Dohura, Katsumi; Ironside, James W; Iwaki, Toru. Increased clusterin (apolipoprotein J) expression in human and mouse brains infected with transmissible spongiform encephalopathies. Acta Neuropathologica (Berl). 2002 Mar; 103(3): 199-208. ISSN: 0001-6322.
     Abstract: Clusterin (apolipoprotein J), a multifunctional protein involved in amyloidogenesis in Alzheimer's disease, was studied immunohistochemically in both human transmissible spongiform encephalopathies (TSEs) and a mouse model of human TSE. Clusterin immunoreactivity was co-localized with plaque-type deposits but not with punctate-type prion protein (PrP) deposits in human TSEs. On the other hand, clusterin-positive astrocytes were readily demonstrated in the regions of punctate PrP deposits, but not around plaque PrP deposits despite the presence of surrounding astrocytes. Clusterin expression in astrocytes was not disease specific, but the punctate immunoreactivity for clusterin was more prominently demonstrated in TSEs with punctate PrP deposits. Serial analysis in the mouse model of human TSE revealed that clusterin expression in astrocytes was enhanced in the lesions with punctate-type PrP deposits during the disease progression. Thus, the induction of clusterin expression in astrocytes could be more enhanced by punctate-type PrP deposits than by plaque-type deposits. The clusterin molecules co-localized in plaque PrP deposits might be derived not from surrounding astrocytes but from other resources such as cerebrospinal fluid and blood plasma, both of which contain clusterin in significant amounts. Taken together with previously reported findings of the anti-amyloidogenic property in clusterin, our findings suggest that clusterin may be induced as one of the important molecules participating in the neurodegeneration caused by abnormally deposited PrP.
     Descriptors: protein analysis, immunohistochemical assay, TSE's mouse model, plaque PrP deposites, neurodegeneration process.

140. Scholz, Roland. 25 Thesen gegen die Behauptung, BSE und vCJK seien oral ubertragbare Infektionskrankheiten und BSE gefahrde die menschliche Gesundheit. [25 theses against the assertion that BSE and vCJD are orally transmissible infectious diseases and endanger human health]. Deutsche Medizinische Wochenschrift. 2002 Feb 15; 127(7): 341-3. ISSN: 0012-0472. In German.
     NAL call no. 448.8 D48
     Descriptors: Nv Creutzfeldt-Jakob Disease, bovine spongiform spongiform encephalopathy, transmission theories, cattle, epidemiology, meat products, human health food risks, risk factors.

141. Schreuder, B.E.C.; Wever, C.J.G. Waar komt BSE in Nederland vandaan? [The possible origin of BSE in the Netherlands.] Tijdschrift voor Diergeneeskunde. 2002, 127: 2, 40-50; 29 ref. ISSN: 0040-7453. In Dutch with an English summary.
     Abstract: It is only in the last 5 years that the Netherlands has been confronted with cases of bovine spongiform encephalopathy (BSE). The cases diagnosed to date have not been clearly linked to imports from the United Kingdom. This article describes the various possible explanations for the Dutch cases. The risk factors involved, have either a connection with imported BSE, local origin of BSE, or both. These factors can also be divided into introductory risk and propagation risk, terms that were also used in an EU risk assessment study. Research at ID-Lelystad since the early 1990s and at IKC-Ede has tried to assess the relative importance of the various risk factors, the results of which are discussed in this paper. The paper does not deal with the specifics of the cases diagnosed to date, because of the absence of an in-depth epidemiological investigation, but provides a general assessment of the risk factors that might have played a role. Important factors have been, in addition to the initial imports of cattle and meat and bone-meal from the UK, the continuing imports from other countries with covert BSE and the cross-contamination within the animal feed production lines. Emphasis is on the period of the early and mid-1990s, the period in which most calves with diagnosed BSE were born.
     NAL call no. 41.8 T431
     Descriptors: bovine spongiform encephalopathy, BSE, history of disease introduction, animal feeds, Netherlands, importance of risk factors leading to disease introduction, importation of infected animals, animal feed formulations.

142. Schulz-Schaeffer, Walter J. BSE und variante CJK. Von den Schwierigkeiten, ein neues Krankheitsprinzip zu etablieren. [BSE and variant CJD: about the difficulties to establish a new pathogenetic principle.] Deutsche Medizinische Wochenschrift 2002 Feb 15; 127(7): 344-6. ISSN: 0012-0472. In German.
     NAL call no. 448.8 D48
     Descriptors: bovine spongiform encephalopathy, cattle, consumer food safety, Europe, epidemiology, NvCreutzfeldt-Jakob Disease, cattle, hamsters, mice, primates, prions, risk factors, scrapie, time factors.

143. Schutt-Abraham, Ingrid. BSE-Praventivmassnahmen bei der Schlachtung von Rindern. [Measures preventing BSE-contamination during the slaughter of cattle.] Berliner und Munchener Tierarztliche Wochenschrift. 2002 Mar-Apr; 115(3-4): 125-30. ISSN: 0005-9366. In German.
     Abstract: SRM-regulations and the prohibition of pithing have removed major risks of spreading BSE-infection. Traditional slaughter technology, especially captive bolt stunning, head handling and carcass splitting nevertheless still provide non-negligible risks for contamination with the BSE-agent if present in cattle, and should therefore be replaced by safer techniques. However, alternative methods like electrical stunning or removal of the spinal cord prior to splitting the carcass cannot yet be considered a reliable and practical option. Surface contamination could be prevented altogether by abandoning the practice of carcass splitting and by removing the vertebral column while still connected to the head, although this would result in disadvantages for post mortem inspection.
     NAL call no. 41.8 B45
     Descriptors: cattle, slaughter, captive bolt, head handling, carsass splitting, contamination with CNS tissue, alternative methods, spinal cord removal.

144. Sethi, Shneh; Lipford, Grayson; Wagner, Hermann; Kretzschmar, Hans. Postexposure prophylaxis against prion disease with a stimulator of innate immunity. Lancet. 2002 Jul 20; 360(9328): 229-30 ISSN: 0140-6736.
     Abstract: The absence of an immune response to prions--the infectious agents of scrapie, bovine spongiform encephalopathy (BSE), and Creutzfeldt-Jakob disease--might be related to the fact that these agents do not contain nucleic acids. We aimed to use CpG oligodeoxynucleotides, which have been shown to stimulate innate immunity, as a form of postexposure prophylaxis in mice. We inoculated healthy mice with brain homogenates from mice infected with the RML scrapie prion, and then injected CpG oligodeoxynucleotides. This postexposure prophylaxis with CpG oligodeoxynucleotides resulted in 38% longer survival times than treatment with saline (p<0.0001), or even longer after repeated application. The explanation for this finding remains to be elucidated, but the most likely is stimulation of TLR9-expressing cells of the innate immune system such as macrophages, monocytes, and dendritic cells. CpG oligodeoxynucleotides have not been shown to have adverse effects to human health and could therefore be considered as a therapeutic choice in postexposure prophylaxis.
     NAL call no. 448.8 L22
     Descriptors: infectious agents, scrapie, bovine spongiform encephalopathy (BSE), and Creutzfeldt-Jakob disease, mouse model, CpG oligodeoxynucleotides, stimulation of immunity.

145. Shaked, Yuval; Engelstein, Roni; Gabizon, Ruth. The binding of prion proteins to serum components is affected by detergent extraction conditions. : Journal of Neurochemistry. 2002 Jul; 82(1): 1-5 ISSN: 0022-3042.
     Abstract: As many GPI anchored proteins, PrP(C) and its abnormal conformer PrP(Sc), are inserted into membrane microdomains known as rafts. Upon raft disruption, PrP(C) becomes soluble, while PrP(Sc) aggregates into insoluble structures. It was recently published that, as opposed to PrP(C), PrP(Sc), as well as its protease resistant core PrP27-30, can bind specifically to plasminogen and other serum components. These findings were suggested to have important physiological implications in transmissible spongiform encephalopathies (TSE) diagnosis and pathogenesis. In this work, we show that the binding of PrP(Sc) or PrP 27-30 to serum proteins occurs only at specific detergent combinations, in which disease associated PrPs are present in aggregated structures. At detergent conditions in which rafts are intact, it is actually PrP(C.) that binds to blood proteins, albeit not directly, but through neighboring rafts components. Our results therefore indicate that the binding of PrP(Sc) to blood components has no physiological relevance.
     NAL call no. QP351 J6
     Descriptors: raft disruption, PrPSc, factors affecting transmissibility, diagnosis, and pathogenesis, serum protein binding, affects of detergent combinations, physiological relevance.

146. Simak, Jan; Holada, Karel; D'Agnillo, Felice; Janota, Jan; Vostal, Jaroslav G. Cellular prion protein is expressed on endothelial cells and is released during apoptosis on membrane microparticles found in human plasma. Transfusion. 2002 Mar; 42(3): 334-42 ISSN: 0041-1132.
     Abstract: BACKGROUND: Blood and plasma of animals experimentally infected with transmissible spongiform encephalopathies (TSEs) can transmit TSE infection by transfusion. A conformational isoform of prion protein (PrPsc) is believed to be the TSE-infectious agent that propagates by converting the cellular prion protein (PrPc) to additional molecules of PrPsc. In orally infected animals, PrPsc accumulates in intestinal endothelial cells. In blood, two thirds of PrPc resides in plasma, but its source is not known. STUDY DESIGN AND METHODS: The expression of PrPc in cultured human umbilical vein endothelial cells (HUVECs) was studied using flow cytometry, immunoblotting, and RT-PCR. Flow cytometry was used to characterize endothelial membrane microparticles (MPs) in cell culture supernatants and in normal human plasma. RESULTS: HUVECs and bovine aorta endothelial cells express PrPc. The number of surface PrPc molecules per cell in HUVECs was 58,000 +/- 2,800. The induction of apoptosis in HUVECs led to a marked release of membrane MPs (60,000-80,000 MPs/10(3) cells) that expressed PrPc and other endothelial antigens. The presence of endothelial cell-derived MPs expressing PrPc was demonstrated in platelet-free human plasma. CONCLUSION: Endothelial cell apoptosis is associated with the release of PrPc-positive MPs. These MPs contribute to the PrPc pool in plasma and may have a role in disseminating TSE infectivity in blood.
     Descriptors: experimental infections, TSEs, transmissible spongiform encephalopathies, PrPsc, cultured human umbilical vein endothelial cells, membrance microparticles carriers.

147. Singh, Neena; Gu, Yaping; Bose, Sharmila; Kalepu, Sudheera; Mishra, Ravi Shankar; Verghese, Susamma. Prion peptide 106-126 as a model for prion replication and neurotoxicity. Frontiers of Bioscience Computer File: a Journal and Virtual Library. 2002 Apr 1; 7: a60-71. ISSN: 1093-4715.
     Abstract: Prion diseases or transmissible spongiform encephalopathies are neurodegenerative disorders that are genetic, sporadic, or infectious. The pathogenetic event common to all prion disorders is a change in conformation of the cellular prion protein (PrPC) to the scrapie isoform (PrPSc), which, unlike PrPC, aggregates easily and is partially resistant to protease digestion. Although PrPSc is believed to be essential for the pathogenesis and transmission of prion disorders, the mechanism by which PrPSc deposits cause neurodegeneration is unclear. It has been proposed that in some cases of prion disorders, a transmembrane form of PrP, termed CtmPrP may be the mediator of neurodegenerative changes rather than PrPSc per se. In order to understand the underlying cellular processes by which PrPSc mediates neurodegeneration, we have investigated the mechanism of neurotoxicity by a beta-sheet rich peptide of PrP in a cell model. We show that exposure of human neuronal cell lines NT-2 and M17 to the prion peptide 106-126 (PrP106-126) catalyzes the aggregation of endogenous cellular prion protein (PrPC) to an amyloidogenic form that shares several characteristics with PrPSc. Intracellular accumulation of these PrPSc-like forms upregulates the synthesis of CtmPrP, which is proteolytically cleaved in the endoplasmic reticulum and the truncated C-terminal fragment is transported to the cell surface. In addition, we have isolated mutant NT-2 and neuroblastoma cells that are resistant to toxicity by PrP106-126 to facilitate further characterization of the biochemical pathways of PrP106-126 neurotoxicity. The PrP106-126-resistant phenotype of these cells could result from aberrant binding or internalization of the peptide, or due to an abnormality in the downstream pathway(s) of neuronal toxicity. Thus, our data suggest that PrPSc aggregation occurs by a process of 'nucleation' on a pre-existing 'seed' of PrP. Furthermore, the PrP106-126-resistant cells reported here will provide a unique opportunity for identifying the cellular and biochemical pathways that mediate neurotoxicity by PrPSc.
     Descriptors: Prion diseases, transmissible spongiform encephalopathies, PrPSc, human neuronal call lines Nt-2, M17, PrP 106-126, process is nucleation on a pre-existing seed.

148. Somerville, R.A.; Oberthur, R.C.; Havekost, U.; Macdonald, F.; Taylor, D.M.; Dickinson, A.G. Characterization of thermodynamic diversity between transmissible spongiform encephalopathy agent strains and its theoretical implications. J Biol Chem. Mar 29, 2002. v. 277 (13) p. 11084-11089. ISSN: 0021-9258
     DOI: 10.1074/jbc.M111766200
     Abstract: Some transmissible spongiform encephalopathy (TSE) (or "prion") strains, notably those derived from bovine spongiform encephalopathy, are highly resistant to total inactivation by heat. When three TSE strains derived from sheep with scrapie were heated, little inactivation took place at low temperatures, but at higher temperatures, considerable inactivation occurred. The temperature at which substantial inactivation first occurred varied according to TSE strain, and it was calculated to be 70 degrees C for the 22C strain, 84 degrees C for ME7, and 97 degrees C for 22A by fitting the data to a model based on competition between a destructive and a protective reaction. However, PrP(Sc) from mice infected with a range of TSE strains retained similar resistance to proteinase K digestion after heating to below or above these temperatures, showing that the properties of PrP(Sc) responsible for proteinase resistance do not correlate with those conferring thermostability on the TSE agent. The simplest explanation of these data is that the causal agent contains a macromolecular component that is structurally independent of the host, that it varies covalently between TSE strains, and that it is protected by other macromolecular components. The model is in accord with the virino hypothesis, which proposes a host-independent informational molecule protected by the host protein PrP.
     NAL call no. 381 J824
     Descriptors: TSE, scrapie agents, strains, isolation, sheep, strain differences, heat inactivation, temperature effects, infectivity, mouse model, glycoproteins, proteolysis, animal prion proteins.

149. Soto, Claudio; Saborio, Gabriela P.; Anderes, Laurence. Cyclic amplification of protein misfolding: Application to prion-related disorders and beyond. Trends in Neurosciences. August, 2002; 25 (8): 390-394.
     DOI: 10.1016/S0166-2236(02)02195-1
     NAL call no. RC321 T74
     Descriptors: methods, amplify cyclically misfolded proteins in vitro, PMCA, potential diagnostic technique, transmissible spongiform encephalopathies, new technology, applications.

150. Spraker, T.R.; Zink, R.R.; Cummings, B.A.; Wild, M.A.; Miller, M.W.; O'Rourke, K.I. Comparison of histological lesions and immunohistochemical staining of proteinase-resistant prion protein in a naturally occurring spongiform encephalopathy of free-ranging mule deer (Odocoileus hemionus) with those of chronic wasting disease of captive mule deer. Veterinary Pathology. 2002 January; 39(1): 110-119 ISSN: 0300-9858.
     NAL call no. 41.8 P27
     Descriptors: mule deer, comparison between captive and free ranging, PrPRES, chronic wasting disease, TSE, histological lesions and immunohistochemical staining.

151. Spraker, T.R.; O' Rourke, K. I.; Balachandran, A.; Zink, R.R.; Cummings, B.A.; Miller, M.W.; Powers, B.E. Validation of monoclonal antibody F99/97.6.1 for immunohistochemical staining of brain and tonsil in mule deer (Odocoileus hemionus) with chronic wasting disease. Journal of Veterinary Diagnostic Investigation. 2002, 14: 1, 3-7; 18 ref.
     NAL call no. SF774.J68
     Descriptors: deer, transmissible spongiform encephalopathies, brain and tonsil tissue staining, diagnosis, chronic wasting disease, immunohistochemical staining method, wildlife,Colorado, Montana.

152. Staufenbiel, Rudolf; Hamalainen, Mira. Zur klinischen Diagnostik der BSE. [The clinical diagnosis of BSE.] Berliner und Munchener Tierarztliche Wochenschrift. 2002 Mar-Apr; 115(3-4): 99-105 ISSN: 0005-9366. In German.
     Abstract: Diagnosis of Bovine spongiform encephalopathy (BSE) is confirmed by specified laboratory methods on brain material. On the other hand clinical signs of manifest BSE are quite obvious. The first part of this paper describes case histories, clinical signs, laboratory findings and the most common differential diagnoses. On the basis of the data of actual prevalence in Germany, the role of clinical examination in eradication of BSE is dealt in the second part. Clinical diagnosis is a very sensitive and specific method when there is a high prevalence. According to the data from December 2000 to November 2001 prevalence in Germany was beyond 1 BSE case per 100,000 cattle or 3 cases per 100,000 cows. This very low prevalence decreases rapidly sensitivity and specificity of the diagnosis made by clinical examination. Therefore the main focus of field-diagnostics has to be laid on specified laboratory diagnostic methods. On the other hand prevalence of BSE-positive cattle is distinctly higher in the group of animals slaughtered in cases of illness or emergency than in cattle slaughtered on the regulatory bases. Nevertheless every veterinary practitioner should be aware of the clinical picture of BSE, clinical examination-routine and differential diagnosis, because occurrence of BSE is still possible in any dairy herd. At the moment it is not possible to make any statement if eradication of BSE can be reached in future.
     NAL call no. 41.8 B45
     Descriptors: cattle, bovine spongiform encephalopathy, case histories, clinical signs, lab findings, differential diagnoses, control and eradication.

153. Stewart, Gordon T.More on BSE/vCJD. Journal of the Royal Society of Medicine 2002 Feb; 95(2): 112. ISSN: 0141-0768.
     NAL call no. 448.9 R814
     Descriptors: NvCreutzfeldt Jakob syndrome, epidemiology, bovine spongiform encephalopathy, cattle, humans transmission.

154. Sugaya, Makoto; Nakamura, Koichiro; Watanabe, Takahiro; Asahina, Akihiko; Yasaka, Nami; Koyama, Yoh Ichi; Kusubata, Masashi; Ushiki, Yuko; Kimura, Kumiko; Morooka, Akira; Irie, Shinkichi; Yokoyama, Takashi; Inoue, Keiichi; Itohara, Shigeyosi; Tamaki, Kunihiko. Expression of cellular prion-related protein by murine Langerhans cells and keratinocytes. Journal of Dermatological Science. February, 2002; 28 (2): 126-134. ISSN: 0923-1811.
     DOI: 10.1016/S0923-1811(01)00160-8
     Abstract: Transmissible spongiform encephalopathies are characterized by the accumulation of a proteinase-resistant isoform of the cellular prion-related protein (PrPc) within the central nervous system (CNS). The accumulation of scrapie-associated PrP (PrPSc) within cells of the lymphoreticular system prior to its accumulation in the CNS is regarded as important for the development of neurological diseases after peripheral inoculation. Little, however, is known as to which cells are the targets for peripheral inoculation. Here, the presence of PrPc on murine Langerhans cells (LC), dendritic cells in the skin and mucosa, and keratinocytes (KC) is demonstrated by immunohistochemical staining, Western-blotting and FACS analysis. The expression of PrPc mRNA in freshly purified LC and KC was also detected by reverse transcriptase-polymerase chain reaction. The expression of PrPc on LC was slightly increased during culture. These data suggest that LC and KC may be the targets for peripheral infection with prions.
     Descriptors: Keratinocytes metabolism, Langerhans cells metabolism, phosphoprotein phosphatase metabolism, base sequence, gene expression, immunohistochemistry, mouse model inbred-BALB-C, inbred-C57B, knockout mice, phosphoprotein-phosphatase genetics, prion disease genetics, prion diseases metabolism, transmissible spongiform encephalopathies.

155. Supattapone, Surachai; Nishina, Koren; Rees, Judy R. Pharmacological approaches to prion research. Biochemical Pharmacology. 2002 Apr 15; 63(8): 1383-8 ISSN: 0006-2952
     Abstract: The "protein-only" mechanism by which infectious agents of prion diseases such as Creutzfeldt-Jakob disease and bovine spongiform encephalopathy replicate remains undetermined. The identification of several distinct classes of prion inhibitors has created an opportunity to investigate the mechanism of prion formation using pharmacological tools. These new inhibitors include substituted tricyclic derivatives, tetrapyrrole compounds, cysteine protease inhibitors, branched polyamines, and specific antibodies. Each inhibitor class contains at least one active compound that inhibits prion propagation in cell culture at sub-micromolar concentrations and several structurally related, inactive compounds. Work with branched polyamines and specific antibodies has already provided insight into the kinetics and cell biology of endogenous prion clearance mechanisms. Other anti-prion compounds do not appear to bind directly to the prion protein. Detailed investigation of the mechanism of drug action of these compounds may lead to the identification of novel prion propagation factors.
     NAL call no. 396.8 B52
     Descriptors: prion diseases, infectious agents, CJD, BSE, bovine spongiform encephalopathy, tricyclic derivatives, tetrapyrroles, cysteine protease inhibitors, branched polyamines, specific antibodies, drug actions.

156. Sy, Man Sun; Gambetti, Pierluigi; Wong, Boon Seng. Human prion diseases. Medical Clinics of North America. May, 2002; 86 (3): 551-571.
     NAL call no. RC660 S95 F&N B-4343
     Descriptors: BSE, Creutzfeldt Jakob Disease, NvCreutzfeldt Jakob Disease, kuru, other transmissible or genetic spongiform encephalopathies.

157. Takekida, Kaori; Kikuchi,Yutaka; Yamazaki, Takeshi; Horiuchi, Motohiro; Kakeya, Tomoshi; Shinagawa, Morikazu; Takatori, Kosuke; Tanimura, Akio; Tanamoto, Ken-Ichi; Sawada, Jun ichi. Quantitative analysis of prion protein by immunoblotting. Journal of Health Science. June, 2002; 48 (3): 288-291.
     URL: http://jhs.pharm.or.jp/data/48(3)/48_288.htm
     Descriptors: TSE, transmissible spongiform encephalopathy, PrPSc, quantitiative analysis technique, immunoblotting, densitometry data, bovine PrPC, deglycosylated forms.

158. Taylor, David. Inactivation of the BSE agent. Comptes Rendus Biologies. Janvier, 2002; 325 (1): 75-76.
     DOI: 10.1016/S1631-0691(02)01386-0
     NAL call no. Q2 C6
     Descriptors: BSE, resistant to inactivation procedures, sodium hypochlorite solutions and autoclaving combined, mouse passaged agent, acid treated bone.

159. Tibayrenc, Michel; Mas, Coma Santiago; Piffaretti, Jean Claude; Struelens, Marc. The European Centre for Infectious Diseases: An adequate response to the challenges of bioterrorism and major natural infectious threats. Infection Genetics and Evolution. May, 2002; 1 (3): 179-181.
     DOI: 10.1016/S1567-1348(02)00035-7
     Descriptors: government center, Europe, organization to address invasive diseases such as bovine spongiform encephalopathy, other zoonotic diseases.

160. Tompa, Peter; Tusnady, Gabor E; Friedrich, Peter; Simon, Istvan The role of dimerization in prion replication. Biophysical Journal. April, 2002; 82 (4): 1711-1718.
     URL: http://www.biophysj.org/cgi/content/abstract/82/4/1711
     Descriptors: prion disease, conformational transition, dimerization role in prion replication, intramolecular disulfide bridge, theory to explain prion conformational changes.

161. Tuzi, Nadia L.; Gall, Elaine; Melton, David; Manson, Jean C. Expression of doppel in the CNS of mice does not modulate transmissible spongiform encephalopathy disease. Journal of General Virology. 2002 Mar; 83(Pt 3): 705-11. ISSN: 0022-1317.
     Abstract: Late onset ataxia reported in three independently derived PrP null lines of mice has been attributed to the overexpression of the doppel protein in the CNS of these mice rather than to the loss of PrP. The central role of PrP in the transmissible spongiform encephalopathies (TSEs), the proximity of the gene which encodes doppel (Prnd) to the PrP gene (Prnp) and the structural similarity shared by PrP and doppel have led to the proposition that ataxia which develops during TSE disease could, in part, be due to doppel. In order to address this hypothesis, we have crossed our two inbred lines of PrP null mice, which either express (RCM) or do not express (NPU) the Prnd gene in the CNS, with mice expressing two Prnp(a[108F189V]) alleles of the PrP gene. We have found that the TSE infection does not influence the level of expression of Prnd in the CNS at the terminal stages of disease. Moreover, we have demonstrated that the level of expression of Prnd in the CNS has no influence on the incubation period, vacuolar pathology nor amount or distribution of deposition in the brains of the TSE-infected mice. Doppel has therefore no apparent influence on the outcome of TSE disease in transgenic mice, suggesting it is unlikely to be involved in the naturally occurring TSE diseases in other species.
     NAL call no. QR360.A1J6
     Descriptors: PrP, doppel protein, mouse models, TSE.

162. VandeVondele, Joost; Colombo, Maria Carola; Laio, Alessandro; Guidoni, Leonardo; Rothlisberger, Ursula. QM/MM study of the copper binding site of prion protein. Biophysical Journal. January, 2002; 82 (1 Part 2): 487a. 46th Annual Meeting of the Biophysical Society, San Francisco, California, USA, February 23-27, 2002
     NAL call no. 442.8 B5238
     Descriptors: prion proteins, molecular binding structure, copper, QM/MM study.

163. Van't Hooft, A. J.G. BSE en de soortspecifieke barriere. [BSE and species specificity.] Tijdschrift voor Diergeneeskunde. 2002 Mar 1; 127(5): 175 ISSN: 0040-7453. In Dutch.
     NAL call no. 41.8 T431
     Descriptors: Creutzfeldt Jakob syndrome, etiology, bovine spongiform encephalopathy, transmission, species specificity.

164. Wahlstrom, Helene; Elvander, Marianne; Engvall, Anders; Vagsholm, Ivar Risk of introduction of BSE into Sweden by import of cattle from the United Kingdom. Preventive Veterinary Medicine. 25 June, 2002; 54 (2): 131-139.
     DOI: 10.1016/S0167-5877(02)00019-3
     NAL call no. SF601.P7
     Descriptors: cattle, BSE risks, transported cattle from UK to Sweden, 1980, surveillance system.

165. Wiemer, Udo. Veterinarrechtliche Schutzmassnahmen im Hinblick auf BSE. [Protective measures taken with regard to BSE under veterinary law]. Berliner und Munchener Tierarztliche Wochenschrift. 2002 Mar-Apr; 115(3-4): 134-9 ISSN: 0005-9366. In German.
     Abstract: BSE was established for the first time in 1986 as a separate disease complex. Since 1989 measures to protect human and animal health have been adopted at Community level and under German law. The article describes the most important provisions governing the prevention, control and eradication of TSE. It addresses in detail the ban on feeding, active monitoring of BSE, active monitoring of small ruminants, measures taken after the detection of BSE, the removal and destruction of specified risk material and briefly addresses trade bans and restrictions.
     NAL call no. 41.8 B45
     Descriptors: BSE, transmissible spongiform encephalopathies, prevention, control, eradication, animal feeds, small ruminants, trade base and restructions.

166. Will, R.G. Variant Creutzfeldt-Jakob disease - How new is new?. Journal of Neurology Neurosurgery and Psychiatry. March, 2002; 72 (3): 285-286.
     URL: http://jnnp.bmj.com/cgi/content/extract/72/3/285
     Descriptors: BSE, bovine spongiform encechalopathy, NvCJD, pathogenesis.

167. Wrathall, A.E.; Brown, K.F.D.; Sayers, A.R.; Wells, G.A.H.; Simmons, M.M.; Farrelly, S.S.J.; Bellerby, P.; Squirrell, J.; Spencer, Y.I.; Wells, M.; Stack, M.J. Studies of embryo transfer from cattle clinically affected by bovine spongiform encephalopathy (BSE). Veterinary Record. Mar 23, 2002. v. 150 (12) p. 365-378. ISSN: 0042-4900
     NAL call no. 41.8 V641
     Descriptors: cattle embryo transfer, bovine spongiform encephalopathy, disease transmission, washing, semen, fertilizing ability, gametogenesis, prion proteins, genotypes, infectivity, UK.

168. Wuthrich, Kurt; Calzolai, Luigi; Guntert, Peter; Luhrs, Thorsten; Lysek, Dominik; Schorn, Christian; Von Schroetter, Christine; Zahn, Ralph. Species variation of the three-dimensional prion protein structure in the cellular form. Biophysical Journal. January, 2002; 82 (1 Part 2): 169a. 46th Annual Meeting of the Biophysical Society, San Francisco, California, USA, February 23-27, 2002
     NAL call no. 442.8 B5238
     Descriptors: prion proteins, protein structions and conformation, species variation.

169. Zaaijer, H. L. Interpretatie van de toename van boviene spongiforme encefalopathie buiten Groot-Brittannie. [Interpretation of the increase in bovine spongiform encephalopathy outside Great Britain.] Nederlands Tijdschrift voor Geneeskunde 2002 Apr 20; 146(16): 748-50. ISSN: 0028-2162. In Dutch.
     Abstract: Outside Great Britain, the number of clinical cases of bovine spongiform encephalopathy (BSE) seems to be rising. It is unclear whether this increase is real, or whether it is caused by improved recognition and improved registration of BSE. A strict and independent control of the implementation of measures intended to keep human food free of the BSE agent is imperative.
     Descriptors: bovine spongiform encephalopathy, cattle, consumer food safety, Europe, epidemiology, Great Britian, control measures.

170. Zentek, J.; Oberthur, R.C.; Kamphues, J.; Kreienbrock, L.; Flachowsky, G.; Coenen, M.. Futtermittel tierischer Herkunft als mogliche Verbreitungsursache fur die bovine spongiforme Enzephalopathie (BSE) in Deutschland. 2. Mitteilung: Einschatzung des Verbreitungsrisikos uber Mischfutter. [Animal-derived feedstuffs as possible vectors for bovine encephalopathy (BSE) in Germany. Part 2: Assessment of vector risk for compounded feed.] . DTW. Deutsche Tierarztliche Wochenschrift. 2002 Feb: 109 (2): 43-51 ISSN: 0341-6593. In German.
     Abstract: Specific conditions and practices of cattle feeding in Germany have to be taken into account for assessing the risk of feed born transmission of bovine spongiform encephalopathy, especially regarding the situation before the year 2000 when specific directives were introduced for feed production. The present retrospective epidemiological study includes data on feed production and the estimated amount of animal derived feedstuffs for the production of compounded feed for cattle. Risk assessment was performed based on the 'reproduction rate' (R0), that is defined as the estimated number of infections resulting from the processing of brain and spinal cord of BSE affected cattle that is recycled to bovines via feed. Under the conditions as given in Germany until the year 2000 the reproduction rate of BSE via the inclusion of animal derived feedstuffs in compounded feed production for cattle was estimated to be 1.1. Thus, it can be expected that BSE could be reproduced in the system, but with comparatively low efficiency. The expected incidence of BSE should be considerably lower compared to the situation during the 90th in the UK, due to the markedly lower recycling rate of animal protein in cattle feeding. Animal fat could have been a significant factor for BSE transmission due to contamination by proteinaceous brain and spinal cord material during the production process. The relative significance of fat containing feedstuffs for BSE transmission could have been higher in Germany compared to the situation in the UK where meat and bone meal was produced under different conditions and frequently used in higher proportions as an ingredient for compounded feed for ruminants.
     NAL call no. 41.8 D482
     Descriptors: cattle, feeding and feeds, BSE, disease transmission risks, animal derived feed additives, Germany.

171. United States. General Accounting Office. Mad cow disease: improvements in the animal feed ban and other regulatory areas would strengthen U.S. prevention efforts. Report to Congressional Requestors, Washington, D.C.: GAO, January 2002. 59 p.
     URL: www.gao.gov/new.items/d02183.pdf
     Descriptors: Bovine spongiform encephalopathy, animal feed, prevention strategies, public and animal health risks, animal feed ban compliance, probablistic simulation model, detection in imported animal products, economic impacts of the disease, recommendations to USDA and FDA, GAO report.

2001

1. Abbott, A. BSE fallout sends shock waves through Germany. Nature. 2001 Jan 18. 409(6818): 275 ISSN: 0028-0836
   NAL call no. 472 N21
   Descriptors: Bovine spongiform encephalopathy, meat, cattle, consumer product safety, Germany, legislation, food, meat products.

2. Abbott, A. Mad-cow outbreak spurs German drive to combat prion diseases. Nature. 2001 Aug 9. 412(6847): 571-572 ISSN: 0028-0836
   NAL call no. 472 N21
   Descriptors: BSE, Germany, strategies to combat the disease, prion diseases, protective measures.

3. Adam, D. Fears rise over BSE infection in UK abattoirs. Nature. 2001 Jun 14. 411(6839): 728 ISSN: 0028-0836
   NAL call no. 472 N21
   Descriptors: Bovine spongiform encephalopathy, slaughter plants, transmission of contaminated material, UK.

4. Adam, D. Catgut sutures--possible BSE risk. Australian Veterinary Journal. 2001 Apr. 79(4): 245-246 ISSN: 0005-0423
   NAL call no. 41.8 Au72
   Descriptors: Bovine spongiform encephalopathy, catgut, transmission, adverse effects, standards, cattle, risk factors.

5. Adam, D. Review blames BSE outbreak on calf feed. Nature. 2001 Aug 2. 412(6846): 467. ISSN: 0028-0836
   NAL call no. 472 N21
   Descriptors: BSE, calf feeding

6. Aguzzi A. Bovins, humains: quelles connexions? [Cattle and people: what are the connections?] Biofutur. 2001, No. Hors Serie, 26-29; 11 ref. ISSN: 0294-3506. In French.
   NAL call no. TP248.13 B565
   Descriptors: asymptomatic infections, bovine spongiform encephalopathy, brain, carrier state, cattle diseases, NvCreutzfeldt-Jakob disease, digestive tract, routes of disease transmission, lymphocytes, prion diseases.

7. Aguzzi, A.; Montrasio, F.; Kaeser, P.S. Prions: Health scare and biological challenge. Nature Reviews: Molecular Cell Biology, 2001 vol. 2, no. 2, pp. 118-126 ISSN: 1471-0072
   Descriptors: yeasts, central nervous system, prion proteins, NvCreutzfeldt-Jakob-disease, BSE, bovine spongiform encephalopathy, cattle, immune system lymphocytes and follicular dendritic cells, transport of prions.

8. Andersson I.; Svendsen L.S.; Gustafsson B. Vad hander med lantbrukets husdjur i katastrofsituationer? [What happens to farm animals in emergency situations?] Svensk Veterinartidning. 2001, 53: 6, 333-339; 11 ref. ISSN: 0346-2250. In Swedish.
   NAL call no. 41.9 SV23
   Descriptors: animal welfare, bovine spongiform encephalopathy, emergencies, foot and mouth disease, livestock.

9. Anil, M.H.; Love, S.; Helps, C.R.; McKinstry, J,L.; Brown, S.N.; Philips, A.; Williams, S.; Shand, A.; Bakirel, T.; Harbour, D. Jugular venous emboli of brain tissue induced in sheep by the use of captive bolt guns. Veterinary Record. 2001, 148: 20, 619-620; 10 ref. ISSN: 0042-4900
   NAL call no. 41.8 V641
   Descriptors: sheep, experimental procedure, jugular sampling, pre- and post-stunning, stunning, electrical stunning, emboli of CNS tissue compared to stunning methods.

10. Axelsson, S. The basic reality of mind and spongiform diseases. Medical Hypotheses. November, 2001; 57 (5): 549-554. ISSN: 0306-9877
    Descriptors: prions, Creutzfeldt-Jakob Disease, bovine spongiform encephalopathy, Alzheimer's disease, chirality, cholinergic system, pathophysiology, beef products.

11. Bachmann, M.F. Complements for mad-cow disease. Trends in Immunology 2001 Jun. 22(6): 297 ISSN: 1471-4906
    NAL call no. QR180 I56
    Descriptors: Bovine spongiform encephalopathy, immunology, complement immunology, cattle.

12. Balter, M. Infectious diseases. Is BSE in sheep a no-brainer? Science. 2001 Oct 26. 294(5543): 771
    NAL call no. 470 Sci2
    Descriptors: BSE, scrapie, cattle, sheep, transmissible spongiform encephalopathies, prion typing.

13. Balter, M. Infectious diseases. Uncertainties plague projections of vCJD toll. Science. 2001 Oct 26. 294(5543): 770-771 ISSN: 0036-8075
    NAL call no. 470 Sci2
    Descriptors: BSE, variant Creutzfeldt-Jakob Disease, potential incidence.

14. Balter, M. Origins of BSE: Intriguing clues to a scrapie-mad cow link Science (US) 2001 vol. 292, no. 5518, pp. 827-829 ISSN: 0036-8075
    NAL call no. 470 Sci2
    Descriptors: BSE, feeds formulation, transmissibility, cattle, sheep, scrapie, prion protein, NvCreutzfeldt-Jakob disease, Uk, France, connection between BSE and NvCJD

15. Baron Thierry, G M; Biacabe, Anne-Gaelle. Molecular analysis of the abnormal prion protein during coinfection of mice by bovine spongiform encephalopathy and a scrapie agent. Journal of Virology. Jan. 2001. v. 75 (1) p. 107-114.
    Abstract: Molecular features of the proteinase K-resistant prion protein (PrP res) may discriminate among prion strains, and a specific signature could be found during infection by the infectious agent causing bovine spongiform encephalopathy (BSE). To investigate the molecular basis of BSE adaptation and selection, we established a model of coinfection of mice by both BSE and a sheep scrapie strain (C506M3). We now show that the PrP res features in these mice, characterized by glycoform ratios and electrophoretic mobilities, may be undistinguishable from those found in mice infected with scrapie only, including when mice were inoculated by both strains at the same time and by the same intracerebral inoculation route. Western blot analysis using different antibodies against sequences near the putative N-terminal end of PrP res also demonstrated differences in the main proteinase K cleavage sites between mice showing either the BSE or scrapie PrP res profile. These results, which may be linked to higher levels of PrP res associated with infection by scrapie, were similar following a challenge by a higher dose of the BSE agent during coinfection by both strains intracerebrally. Whereas PrP res extraction methods used allowed us to distinguish type 1 and type 2 PrP res, differing, like BSE and scrapie, by their electrophoretic mobilities, in the same brain region of some patients with Creutzfeldt-Jakob disease, analysis of in vitro mixtures of BSE and scrapie brain homogenates did not allow us to distinguish BSE and scrapie PrP res. These results suggest that the BSE agent, the origin of which remains unknown so far but which may have arisen from a sheep scrapie agent, may be hidden by a scrapie strain during attempts to identify it by molecular studies and following transmission of the disease in mice.
    NAL call no. QR360 J6
    Descriptors: mouse model, scrapie, bovine spongiform encephalopathy, laboratory inoculation, variant differences, identification of strains. proteinase K-resistant prion protein.

16. Bartels, Dennis. Mad cows and market forces. Journal of Human Ecology. May, 2001; 12 (3): 163-170. ISSN: 0970-9274
    Descriptors: bovine spongiform encephalopathy, safe of food, consumer attitudes, British government claims.

17. Beale, A. J. BSE and vCJD: what is the future? Journal of the Royal Society of Medicine 2001 May. 94(5): 207-209 ISSN: 0141-0768
    NAL call no. 448.9 R814
    Descriptors: bovine spongiform encephalopathy, Cruetzfeldt-Jakob Disease, epidemiology, bovine based products.

18. The beef CMO and BSE -- making a bad problem worse. Agra Europe (Brit. edition.) 2001, No. 1937, A-1-A-2. ISSN: 0002-1024
    NAL call no. 286.8 AG3AE
    Descriptors: BSE, agricultural crises, EU beef industry, bovine spongiform encephalopathy, consumption, costs, impacts on international trade, subsidies, surpluses, consumer concerns and declining consumption trends.

19. Beiglbock, C. Die Chronic Wasting Disease (CWD) der Cerviden in Nordamerika -- eine Literaturubersicht. [Chronic Wasting Disease (CWD) in cervids in North America -- a review.] Wiener Tierarztliche Monatsschrift. 2001, 88: 6, 147-152; 30 ref. ISSN: 0043-535X. In German with an English summary.
    NAL call no. 41.8 T345
    Descriptors: etiology, diagnosis, disease control, prevention strategies, epidemiology, pathology, reviews, transmissible spongiform encephalopathy; wasting disease, deer, elk, North America.

20. Belay, E. D.; Gambetti, P.; Schonberger, L. B.; Parchi, P.; Lyon, D.R.; Capellari, S.; McQuiston, J.H.; Bradley, K.; Dowdle, G.; Crutcher, J.M.; Nichols, C. R. Creutzfeldt-Jakob disease in unusually young patients who consumed venison. Archives of Neurology. 2001 Oct. 58(10): 1673-1678 ISSN: 0003-9942
    Abstract: BACKGROUND: Creutzfeldt-Jakob disease (CJD) in humans and chronic wasting disease (CWD) in deer and elk occur in the United States. Recent reports of 3 unusually young patients with CJD who regularly consumed deer or elk meat created concern about the possible zoonotic transmission of CWD. OBJECTIVE: To examine the possible transmission of CWD to humans. PATIENTS: Three unusually young patients (aged 28, 28, and 30 years) with CJD in the United States during 1997-2000. METHODS: We reviewed medical records and interviewed family members and state wildlife and agriculture officials. Brain tissue samples were tested using histopathologic, immunohistochemical, immunoblot, or prion protein gene analyses. MAIN OUTCOME MEASURES: Presence or absence of established CJD risk factors, deer and elk hunting in CWD-endemic areas, and comparison of the evidence for the 3 patients with that of a zoonotic link between new variant CJD and bovine spongiform encephalopathy. RESULTS: None of the patients had established CJD risk factors or a history of travel to Europe. Two patients hunted game animals and 1 was a daughter of a hunter. Unlike patients with new variant CJD, the 3 patients did not have a unique neuropathologic manifestation, clinicopathologic homogeneity, uniformity in the codon 129 of the prion protein gene, or prion characteristics different from those of classic variants. CONCLUSIONS: Although the occurrence of 3 unusually young patients with CJD who consumed venison suggested a possible relationship with CWD, our follow-up investigation found no strong evidence for a causal link. Ongoing CJD surveillance remains important for continuing to assess the risk, if any, of CWD transmission to humans.
    Descriptors: chronic wasting disease, zoonotic transmission concerns, case study, NvCreutzfeldt-Jakob disease infection, deer and elk meat, 3 young patients, codon 129.

21. Bergmann, W.; Beringer, H. Kupfermangel, ein moglicher BSE-auslosender Faktor? [Copper deficiency -- a potential factor in BSE?] Journal of Plant Nutrition and Soil Science. 2001, 164: 2, 233-235; 13 ref. ISSN: 1436-8730. In German with an English summary.
    NAL call no. QK867 J68
    Descriptors: BSE, normal and pathogenic prions chemistry, bovine spongiform encephalopathy, copper, trace minerals, mineral deficiencies, reviews.

22. Berthelin, Baker C.; Konold, T.; Clifford, D.; Ryder, S.; Bellworthy, S.; Dexter, G.; Brittin, D.; Wood, D.; Simmonds, M.; Bone, G.; Jeffrey, M. Interim observations on the clinical features of Bovine Spongiform Encephalopathy in sheep of the Romney and Suffolk breeds. Research in Veterinary Science. April, 2001; 70 (Supplement A): 28. ISSN: 0034-5288. 55th Annual Conference on Current Topics in Veterinary Science, Scarborough, England, UK, April 09-12, 2001
    NAL call no. 41.8 R312
    Descriptors: BSE, sheep breed susceptibility differences, transmissible spongiform encephalopathies.

23. Bin Kingombe, C.I.; Luthi, E; Schlosser, H; Howald, D; Kuhn, M; Jemmi, T. A PCR-based test for species-specific determination of heat treatment conditions of animal meals as an effective prophylactic method for bovine spongiform encephalopathy. Meat science. Oxford: Elsevier Science Limited. Jan. 2001. v. 57 (1) p. 35-41. Includes references.
    NAL call no. TX373.M4
    Descriptors: polymerase chain reaction, fish meal, pork, ELISA, monitoring, screening, mitochondrial DNA, cytochrome B, genes, cattle, prevention, bovine spongiform encephalopathy.

24. Bindon, B.M.; Jones, N.M. I. Cattle supply, production systems and markets for Australian beef. Australian Journal of Experimental Agriculture. 2001; 41 (7): 861-877. ISSN: 0816-1089
    NAL call no. 23 Au792
    Descriptors: beef cattle, market impacts, BSE, foot and mouth disease, history of industry, 20^th century, brucellosis, tuberculosis, Dick Austen, trade, standards, food safety concerns, new market forces, Australia.

25. Birkett, C.R.; Hennion, R.M.; Bembridge, D.A.; Clarke, M.C.; Chree, A.; Bruce, M.E.; Bostock, C. J. Scrapie strains maintain biological phenotypes on propagation in a cell line in culture. EMBO Journal 2001 Jul 2. 20(13): 3351-3358. ISSN: 0261-4189
    Abstract: Bovine spongiform encephalopathy (BSE) and its human equivalent, variant Creutzfeldt-Jakob disease (vCJD), are caused by the same strain of infectious agent, which is similar to, but distinct from, >20 strains of their sheep scrapie homologue. A better understanding of the molecular strain determinants could be obtained from cells in monoculture than from whole animal studies where different cell targeting is commonly a strain-related feature. Although a few cell types can be infected with different strains, the phenotypes of the emergent strains have not been studied. We have cured the scrapie-infected, clonal SMB cell line with pentosan sulfate, stably re-infected it with a different strain of scrapie and shown that biological properties and prion protein profiles characteristic of each original strain are propagated faithfully in this single non-neuronal cell type. These findings attest to the fact that scrapie strain determinants are stable and host-independent in isolated cells.
    NAL call no. QH506 E46
    Descriptors: BSE, NvCreutzfeldt-Jakob Disease, molecular differences, strains, sheep scrapie, clonal, SMB.

26. Bittante, G. Mercato e tecnica di alimentazione: cosa cambia dopo la BSE. [The market and animal feeding techniques: changes since the BSE crisis.] Informatore Agrario. 2001, 57: 11, 81-85. ISSN: 0020-0689. In Italian.
    NAL call no. 281.8 IN32
    Descriptors: animal feeding, beef, bovine spongiform encephalopathy, disease control, economic impact; feeds, producer prices.

27. Blood-based prion test. Analytical Chemistry. 2001 May 1. 73(9): 252A ISSN: 0003-2700
    NAL call no. 381 J825A
    Descriptors: diagnostic test, screening live mammals, fluorescently labeled peptide from PrP and peptide antibodies, economical preclinical method, CE or size exclusion HPLC.

28. Bol, P. BSE en andere prionziekten. [BSE and other prion diseases] Nederlands Tijdschrift voor Tandheelkunde 2001 Feb. 108(2): 72-73 ISSN: 0028-2200 In Dutch.
    Descriptors: transmissible spongiform encephalopathies.

29. Bonnardiere, C la; la Bonnardiere, C. BSE: le depistage s'organise. [Early detection of bovine spongiform encephalopathy is progressing.] Biofutur. 2001, No. Hors Serie, 52-56; 8 ref. ISSN: 0294-3506. In French.
    NAL call no. TP248.13 B565
    Descriptors: bovine spongiform encephalopathy, diagnostic techniques, Western blot assay, Prionics, brain stem testing. Biorad enzyme assay.

30. Bosch, X. BSE panic spreads to Spain. Nature Medicine. 2001 Feb. 7(2): 138 ISSN: 1078-8956
    Descriptors: bovine spongiform encephalopathy, incidence, disease risks, Spain.

31. Bosch, X. European concern over BSE transmission. JAMA. 2001 Jan 24-31. 285(4): 397-398. ISSN: 0098-7484
    NAL call no. 448.9 AM37
    Descriptors: bovine spongiform encephalopathy, transmissibility, human and animal health risks.

32. Bradley, R.; Rabenau, H.F. (ed.); Cinatl, J. (ed.); Doerr, H.W. Bovine spongiform encephalopathy and its relationship to the new variant form of Creutzfeldt-Jakob disease. Prions: a challenge for science, medicine and public health-system. 2001, 105-144; 48 ref. Published by: S. Karger AG; Basel; Switzerland. ISBN: 3-8055-7124-0
    NAL call no. QR1 C66 v. 7
    Descriptors: BSE, bovine spongiform encephalopathy, NvCreutzfeldt Jakob disease, prion diseases, pathogenesis

33. Bradley, R. A brief overview of bovine spongiform encephalopathy and related diseases including a TSE risk analysis of bovine starting materials used during the manufacture of vaccines for use in humans. Przegl Epidemiol. 2001. 55(3): 387-405 ISSN: 0033-2100
    Descriptors: BSE, transmissible spongiform encephalopathies, bovine based pharmaceutical products, vaccines, public health risks

34. Bradley, R. Obecna sytuacja badan nad pasozowalnymi encefalopatiami gabczastymi. [The current situation of investigation of bovine spongiform encephalopathy] Przeglad Epidemiologiczny. 2001. 55(1 Suppl 2): 37-59 ISSN: 0033-2100. In Polish.
    Descriptors: BSE, Polish situation, epidemiology.

35. Bratberg B.; Benestad S.L.; Schonheit J. "Ny type" scrapie. ["New type" scrapie.] Norsk Veterinaertidsskrift. 2001. 113: 2, 79-80; 4 ref. ISSN: 0332-5741. In Norwegian.
    NAL call no. 41.8 N81
    Descriptors: BSE, bovine spongiform encephalopathy; pathology; scrapie, experimental animals.

36. Brazil, Instituto Brasileiro de Economia. Vaca louca. [Mad cow.] Agroanalysis. 2001, 21: 2, 39-46. ISSN: 0100-4298. In Portuguese.
    Descriptors: BSE, cattle prion diseases, bovine spongiform encephalopathy, pastures grazing systems, cattle feeding, low risk.

37. Bren, L. Trying to keep "Mad Cow Disease" out of U.S. herds. FDA Consumer Rockville, Md. : Food and Drug Administration, Department of Health & Human Services. Mar/Apr 2001. v. 35 (2) p. 12-14. ISSN: 0362-1332
    NAL call no. HD9000.9.U5A1
    Descriptors: bovine spongiform encephalopathy, disease prevention, Creutzfeldt-Jakob Disease. USDA, federal government, disease transmission, surveillance, meat inspection, Federal regulations, meat and livestock industry, ruminant feeding, consumer protection, USA.

38. Brewer, M.S. Bovine spongiform encephalopathy--food safety implications. Adv Food Nutr Res. San Diego : Academic Press, c1989-. 2001. v. 43 p. 265-317. ISSN: 1043-4526
    NAL call no. TX537.A38
    Descriptors: bovine spongiform encephalopathy, food safety, food contamination, rendering, disease transmission, prions, strains, prion diseases, genetic factors, species differences, human health risks, Creutzfeldt-Jakob-Disease, infectivity, bovine-based products, tallow, gelatin, diagnostic techniques, surveillance, disease control, disease prevention, literature reviews, UK, USA.

39. Bromley, D.W. Mad cows, drugged cows, and juggled genes: purpose and necessity in science policy and public opinion. Choices. The Magazine of Food, Farm, and Resources Issues. 2001, No. 2, 6-9. ISSN: 0886-5558
    NAL call no. HD1751.C45
    Descriptors: beef, biosafety, BSE, bovine spongiform encephalopathy, food contamination, food safety, milk, food related public health scares, public, BST.

40. Brown, D.R. BSE: a post-industrial disease? Chemistry and Industry. 2001, No. 3, 73-76; 13 ref. ISSN: 0009-3068
    Descriptors: BSE, etiology, bovine spongiform encephalopathy, NvCreutzfeldt-Jakob disease, prion diseases, cattle, human health risks.

41. Brown, D.R. BSE did not cause variant CJD: An alternative cause related to post-industrial environmental contamination. Medical Hypotheses. November, 2001; 57 (5): 555-560. ISSN: 0306-9877
    Descriptors: prion diseases, Bovine spongiform encephalopathies, NvCreutzfeldt-Jakob Disease, etiology, theories, manganese-rich pollutants.

42. Brown, Paul. Afterthoughts about bovine spongiform encephalopathy and variant Creutzfeldt-Jakob disease. Emerging Infectious Diseases. 2001; 7 (3 Supplement): 598-600. ISSN: 1080-6040.
    NAL call no. RA648.5 E46
    Descriptors: BSE, NvCreutzfeldt-Jakob Disease, issues, new diseases, prion diseases

43. Brown, P. Bovine spongiform encephalopathy and variant Creutzfeldt-Jakob disease. BMJ Clinical Research ed. 2001 Apr 7. 322(7290): 841-844 ISSN: 0959-8138
    Descriptors: Creutzfeldt-Jakob Syndrome, etiology, epidemiology, age distribution, cattle.

44. Brown, P.; Will, R.G.; Bradley, R.; Asher, D.M.; Detwiler, L. Bovine spongiform encephalopathy and variant Creutzfeldt-Jakob disease: background, evolution, and current concerns. Emerging Infectious Diseases 2001 Jan-Feb. 7(1): 6-16 ISSN: 1080-6040
    Abstract: The epidemic of bovine spongiform encephalopathy (BSE) in the United Kingdom, which began in 1986 and has affected nearly 200,000 cattle, is waning to a conclusion, but leaves in its wake an outbreak of human Creutzfeldt-Jakob disease, most probably resulting from the consumption of beef products contaminated by central nervous system tissue. Although averaging only 10-15 cases a year since its first appearance in 1994, its future magnitude and geographic distribution (in countries that have imported infected British cattle or cattle products, or have endogenous BSE) cannot yet be predicted. The possibility that large numbers of apparently healthy persons might be incubating the disease raises concerns about iatrogenic transmissions through instrumentation (surgery and medical diagnostic procedures) and blood and organ donations. Government agencies in many countries continue to implement new measures to minimize this risk.
    NAL call no. RA648.5 E46
    Descriptors: BSE, NvCreutzfeldt-Jakob Disease, UK, human disease risks, tranmissibility concerns, incidence, iatrogenic transmission risks, governmental measures, risk control, epidemiology.

45. Brown, P. The pathogenesis of transmissible spongiform encephalopathy: routes to the brain and the erection of therapeutic barricades. Cellular and Molecular Life Sciences. 2001, 58: 2, 259-265; 51 ref. ISSN: 1420-682X
    NAL call no. QH301 C45
    Descriptors: BSE, bovine spongiform encephalopathy, pathogenesis, NvCreutzfeldt-Jakob disease, treatments, prion diseases, scrapie, spongiform encephalopathy.

46. Brugere-Picoux, J.; Brugere, H. Encephalopathie spongiforme bovine: un tournant dans l'evolution de l'epidemie? [Bovine spongiform encephalopathy: a change in the etiology of the epidemic?] Revue de Medecine Interne, 2001 Aug. 22(8): 693-698 ISSN: 0248-8663. In French.
    Descriptors: BSE, etiology, disease incidence.

47. BSE bij gezelschapsdieren. [BSE in companion animals] Tijdschrift voor Diergeneeskunde 2001 Feb 15. 126(4): 120-121 ISSN: 0040-7453. In Dutch.
    NAL call no. 41.8 T431
    Descriptors: BSE, issues of transmissible spongiform encephalopathies, cats, dogs.

48. Butler, D. Spotlight on scrapie in hunt for sheep BSE. Nature. 2001 Nov 1. 414(6859): 7 ISSN: 0028-0836
    NAL call no. 472 N21
    Descriptors: Bovine spongiform encephalopathy, diagnosis, scrapie, sheep, cattle, Great Britain.

49. Buzby, J.C.; Detwiler, L.R. BSE: anatomy of a crisis. Choices. The Magazine of Food, Farm, and Resources Issues. 2001, No.2, 41-45. ISSN: 0886-5558
    NAL call no. HD1751.C45
    Descriptors: agricultural crises, BSE, bovine spongiform encephalopathy, Creutzfeldt NvJakob disease, economic impact, epidemics, impacts on exports and international trade, meat and livestock industry, US policies, cattle protection.

50. Calavas, D.; Ducrot, C.; Baron, T.; Morignat, E.; Vinard, J.L.; Biacabe, A.G.; Madec, J.Y.; Bencsik, A.; Debeer, S.; Eliazsewicz, M. Prevalence of BSE in western France by screening cattle at risk: preliminary results of a pilot study. Vet Rec. London : The British Veterinary Association. July 14, 2001. v. 149 (2) p. 55-56. ISSN: 0042-4900
    NAL call no. 41.8 V641
    Descriptors: cattle, bovine spongiform encephalopathy, disease prevalence, age risk, France

51. Calza, L.; Manfredi, R.; Chiodo, F. Epidemia di encefalopatia spongiforme bovina e nuova variante della malattia di Creutzfeldt-Jakob nell'uomo. Ultime acquisizioni sulle malattie da prioni. [Epidemics of bovine spongiform encephalopathy and new variant of Creutzfeldt-Jakob disease in humans. Most recent findings on prion disease.] Recenti Progressi in Medicina. 2001 Feb. 92(2): 140-149 ISSN: 0034-1193. In Italian.
    Abstract: Prion diseases have been popularized by extensive media coverage of bovine spongiform encephalopathy (BSE) or "mad cow disease" epidemic, observed in Great Britain since 1986, and new variant Creutzfeldt-Jakob disease (nvCJD), reported for the first time in 1996. In contrast to the classical form of the disease, nvCJD affects younger patients, presents a relatively longer duration of illness and is caused by the same agent as BSE. Evidence from laboratory studies now strongly supports the hypothesis that new variant represents human form of animal disease, linked to exposure, probably through food, to bovine prions. Number of BSE reports in the United Kingdom began to decline in 1993, and has continuously decreased year by year since then, but a great worry spread in European countries in association with new BSE reported cases outside of the Great Britain, and increasing incidence of nvCJD. New epidemiological, clinical, histopathological and experimental data on prion diseases are reviewed, focusing our attention on the possible transmission of prion proteins from animals to humans.
    Descriptors: NvCreutzfeldt-Jakob Disease, BSE, epidemiology, clinical data, UK, European incidence, transmission, human health risks.

52. Caramelli, M; Ru, G; Casalone, C; Bozzetta, E; Acutis, PL; Calella, A; Forloni, G. Evidence for the transmission of scrapie to sheep and goats from a vaccine against Mycoplasma agalactiae. Veterinary Record. 2001, 148: 17, 531-536; 21 ref. ISSN: 0042-4900
    NAL call no. 41.8 V641
    Descriptors: BSE, bovine spongiform encephalopathy, brain, codon 171, cross infection, epidemiology, genotypes, mortality, polymorphism, scrapie, transmission, Mycoplasma agalactiae vaccine as infective source, sheep, goats, brain lesions.

53. Cardone, F.; Pocchiari, M. A role for complement in transmissible spongiform encephalopathies. Nature Medicine 2001 Apr. 7(4): 410-411 ISSN: 1078-8956
    Descriptors: BSE, CJD, blood factors, role in disease formation.

54. Carlier, F. Vers une economie du principe de precaution. [Economy of the precautionary principle.] Biofutur. 2001, No. Hors Serie, 16-20; 7 ref. ISSN: 0294-3506. In French.
    NAL call no. TP248.13 B565
    Descriptors: BSE, traceability measures, bovine spongiform encephalopathy, consumer protection, disease prevention, labeling, meat and meat products, prion diseases, Europe.

55. Carson, C.; McKay, J.S.; Brooks, H.W.; Kelly, D.F.; Stidworthy, M.F.; Wibbelt, G.; Morgan, K.L. Establishment and maintenance of a longitudinal study of bovine spongiform encephalopathy (the ULiSES scheme). Preventive Veterinary Medicine. 2001 Oct 11. 51(3-4): 245-257 ISSN: 0167-5877
    Abstract: This paper addresses the issues of tracing and compliance encountered in setting up and maintaining a UK-wide 5-year observational study of beef cattle. The 5-year prospective study was initiated in 1997 to investigate the occurrence of bovine spongiform encephalopathy (BSE) in a single herd of pedigree Aberdeen Angus cattle, in which BSE had been detected at low prevalence. The study was given the acronym ULiSES (University of Liverpool Spongiform Encephalopathy Scheme). All cattle present on the farm at the start of the scheme were registered as members of the study population (n=320), as were all calves standard histopathological techniques) for the presence of spongiform change. Remaining samples were stored at -70 degrees C for future investigation by alternative tests. At the halfway point of the scheme in October 1999, 75.2% (506/673) of the study population was still alive; 42% (284) of the population was still alive on the study farm and 33% (222) was distributed on other farms throughout the UK. Complete sets of specimens had been recovered from 77% (129/167) of dead animals. All brainstem sections were negative by histopathological examination. No suspect cases of BSE were reported in ULiSES animals. Failure to recover specimens occurred principally in animals which had left the study farm. The main cause of specimen loss was a failure of compliance in a small number of individuals who had purchased large numbers of ULiSES animals, and subsequently slaughtered them without contacting the University. Despite this, farmer compliance was generally high. The ULiSES scheme shows the feasibility of a country-wide longitudinal observational study spanning a period of several years and indicates the large impact of small numbers of non-compliant individuals.
    NAL call no. SF601.P7
    Descriptors: University of Liverpool Spongiform Encephalopathy Scheme, Aberdeen Angus beef cattle, 5 year observational study, post mortem sampling, BSE surveillance.

56. Cashman, N.R. Transmissible spongiform encephalopathies: Vaccine issues. In Developments in Biologicals. Evolving scientific and regulatory perspectives on cell substrates for vaccine development. 2001; (106): 455-461. Fred Brown; Andrew Lewis Jr.; Keith Peden; and Philip Krause Eds S. Karger Publishers Inc., 79 Fifth Avenue, New York, NY, 10003, USA; S. Karger AG, CH 4009, Basel, Switzerland. ISBN 1424-6074
    NAL call no. QR180.3 D4 v. 106
    Descriptors: BSE, transmissible spongiform encephalopathies, vaccine development, techniques, approaches.

57. Caughey, B.; Dobson, C. M. (ed.); Ellis, R. J. (ed.); Fersht, A. R. Prion protein inter-conversions. Protein misfolding and disease. Papers of a Discussion Meeting held at the Royal Society, London, UK, on 23 and 24 February 2000. Philosophical Transactions of the Royal Society of London. Series B, Biological Sciences. 2001, 356: 1406, 197-202; Many ref. ISSN: 0962-8436.
    NAL call no. 501 L84Pb
    Descriptors: BSE, bovine spongiform encephalopathy, NvCreutzfeldt Jakob disease, in vitro models, prion diseases, conversion inhibitors, prion proteins, scrapie

58. Cervenakova, L. The safety of human blood: experimental TSE/prion infectivity studies. Transfusion Clinique et Biologique Journal de la Societe Francaise de Transfusion Sanguine. 2001 Jun. 8(3): 260. ISSN: 1246-7820
    Descriptors: Cattle, blood transfusion, adverse effects, diseases, Creutzfeldt-Jakob Syndrome, transmission, prevention and control, bovine spongiform encephalopathy, Great Britain, human, prion diseases, safety.

59. Cesar, Isigidi K.; Luthi, E.; Schlosser, H.; Howald, D.; Kuhn, M.; Jemmi, T. A PCR-based test for species-specific determination of heat treatment conditions of animal meals as an effective prophylactic method for bovine spongiform encephalopathy. Meat Science. 2001, 57: 1, 35-41; 17 ref. ISSN: 0309-1740
    NAL call no. TX373 M4
    Descriptors: PCR- based assay, screening method, animal meals, bovine spongiform encephalopathy, disease prevention, heat treated meat meal, meat products, pork, polymerase chain reaction.

60. Cesbron, JY; Lemaire, C; Gagnon, J. Comment se propage l'infection. [How the infection is propagated.] Biofutur. 2001, No. Hors Serie, 37-40; 8 ref. ISSN: 0294-3506. In French.
    NAL call no. TP248.13 B565
    Descriptors: BSE, bovine spongiform encephalopathy, mouse scrapie, disease course and models, ileum, immune system, laboratory animals, peripheral nerves, scrapie, comparison.

61. Clarke, A.R.; Jackson, G.S.; Collinge, J.; Dobson, C.M. (ed.); Ellis, R.J. (ed.); Fersht, A.R. The molecular biology of prion propagation. Protein misfolding and disease. Papers of a Discussion Meeting held at the Royal Society, London, UK, on 23 and 24 February 2000. Philosophical Transactions of the Royal Society of London. Series B,-Biological Sciences. 2001, 356: 1406, 185-195; Many ref. ISSN: 0962-8436
    NAL call no. 501 L84Pb
    Descriptors: BSE, bovine spongiform encephalopathy; NvCreutzfeldt Jakob disease; prion protein molecular biology, scrapie, PrPSc, prion propagation.

62. Coe, John E.; Race, Richard E.; Ross, Mary J. Serological evidence for an inflammatory response in murine scrapie. Journal of Infectious Diseases. 15 January, 2001; 183 (2): 185-191. ISSN: 0022-1899.
    NAL call no. 448.8 J821
    Descriptors: transmissible spongiform encephalopathies, murine scrapie experimental model, C57BL10 and IRW mice, serum amyloid P component levels, possible diagnostic test.

63. Collinge, J. Prion diseases of humans and animals: their causes and molecular basis. Annual Review of Neuroscience. 2001. 24: 519-550 ISSN: 0147-006X
    Abstract: Prion diseases are transmissible neurodegenerative conditions that include Creutzfeldt-Jakob disease (CJD) in humans and bovine spongiform encephalopathy (BSE) and scrapie in animals. Prions appear to be composed principally or entirely of abnormal isoforms of a host-encoded glycoprotein, prion protein. Prion propagation involves recruitment of host cellular prion protein, composed primarily of alpha-helical structure, into a disease specific isoform rich in beta-sheet structure. The existence of multiple prion strains has been difficult to explain in terms of a protein-only infections agent, but recent studies suggest that strain specific phenotypes can be encoded by different prion protein conformations and glycosylation patterns. The ability of a protein to encode phenotypic information has important biological implications. The appearance of a novel human prion disease, variant CJD, and the clear experimental evidence that it is caused by exposure to BSE has highlighted the need to understand the molecular basis of prion propagation, pathogenesis, and the barriers limiting intermammalian transmission. It is unclear if a large epidemic of variant CJD will occur in the years ahead.
    NAL call no. QP351.A68
    Descriptors: NvCreutzfeldt-Jakob disease, bovine spongiform encephalopathy, scrapie, abnormal isoforms, prion protein isoforms, prion propagation, pathogenesis, barriers to transmission.

64. Comincini, S.; Foti, M.G.; Tranulis, M.A.; Hills, D.; Di Guardo, G.; Vaccari, G.; Williams, J.L.; Harbitz, I.; Ferretti, L. Genomic organization, comparative analysis, and genetic polymorphisms of the bovine and ovine prion Doppel genes (PRND). Mammalian Genome. 2001 Sep. 12(9): 729-733 ISSN: 0938-8990
    Abstract: The doppel protein (Dpl) is a prion-like protein encoded by the gene PRND, which has been found downstream of the prion gene, PRNP, in human and mouse. This paper describes the isolation and structural organization of the bovine and ovine PRND genes, which are composed of two exons compared with the three of human and mouse. Intergenic distances between PRNP and PRND were covered by means of long-range PCR and found to be 16.8 and 20 kb, in cattle and sheep respectively. The 5' and 3' untranslated regions (UTR) were analyzed to identify transcription regulatory sequences and compared with those from the PRND and PRNP sequences published for other species. Three polymorphisms (R50H, N110H, and R132Q) were revealed in the cattle coding region; two synonymous substitutions (I12I, A26A) were found in sheep. None of the polymorphisms was significantly associated with either Bovine Spongiform Encephalopathy (BSE) in cattle or scrapie in sheep.
    NAL call no. QL738.5.M359
    Descriptors: doppel protein, bovine, ovine PRND genes, structure, isolation, PCR polymorphisms, association with BSE and scrapie.

65. Comincini, S.; Castiglioni, B. M.; Foti, G. M.; Del Vecchio, I.; Ferretti, L. Isolation and molecular characterization of rasfadin, a novel gene in the vicinity of the bovine prion gene. Mammalian Genome. 2001 Feb. 12(2): 150-156 ISSN: 0938-8990
    Abstract: A novel gene, rasfadin (RASSF2) was identified close to the bovine prion gene, and its genomic structure was derived with a combination of exon trapping and RACE. The gene covers at least 28 kb and maps to the same chromosomal region as the prion gene in cattle, sheep, and human. The RASSF2 ORF is composed of 987 base pairs divided into nine exons and shows a high nucleotide (88%) and amino acid similarity (95%) with a previously described human cDNA, KIAA0168. The bovine 3'UTR region is significantly shorter than the human counterpart, but shares with it two highly conserved nucleotide blocks. The expression of the gene was investigated in brain, liver, and spleen. Alternative splicing yields a shorter product in the liver composed of only four exons. Computer analysis showed a highly significant similarity of the rasfadin protein with the Ras association (Ral-GDS/AF-6) domain family 2 and with the afadin family, respectively, for the longer brain/spleen and the shorter liver variants.
    NAL call no. QL738.5.M359
    Descriptors: rasfadin gene, bovine prion gene, exon trapping, RACE, cattle, sheep, humans

66. Cooley, W.A.; Clark, J.K.; Ryder, S.J.; Davis, L.A.; Farrelly, S.S.J.; Stack, M.J. Evaluation of a rapid western immunoblotting procedure for the diagnosis of bovine spongiform encephalopathy (BSE) in the UK. J Comp Pathol. London : W.B. Saunders Company Ltd. July 2001. v. 125 (1) p. 64-70.
    Abstract: Bovine brain tissue samples from 625 UK cattle, clinically suspected as bovine spongiform encephalopathy (BSE) cases, were used in a blind analysis to assess a rapid Western immunoblotting technique (Prionics Check; Prionics AG, Zurich), which detects bovine disease-specific protease-resistant prion protein (PrP(Sc)). By means of statutory histopathological examination, 599 of the 625 cattle were confirmed as BSE cases by the demonstration of spongiform encephalopathy, the remaining 26 being classified as negative. Duplicate samples from the same animals were also examined by electron microscopy for the presence of abnormal brain fibrils (scrapie-associated fibrils; SAFs). The Prionics technique showed a high sensitivity, particularly when compared with the fibril detection test; the detection rates were 99.3% and 92.0% respectively, with histopathology being used as the "gold standard". The false negative results by the Prionics test were possibly related to the sampling procedure. Analysis of 50 BSE-positive samples revealed similar glycoprofiles, the majority of PrP(Sc)isoforms being di-glycosylated protein. The Prionics test also detected PrP(Sc)in the four brain samples from the 26 histopathologically negative animals, apparently reducing the specificity of the test to 84.6%; however, confirmatory positive results in these samples were obtained by demonstrating SAF or by immunohistochemical examination, or both. It was concluded that the Prionics test detected PrP(Sc)in a small percentage (0.64%) of clinically suspected BSE cases showing no spongiform change. Since January 2000, the Prionics Western blot test has been introduced as one of the statutory tests for the diagnosis of clinically suspected BSE and scrapie cases in the UK.
    NAL call no. 41.8 J82
    Descriptors: cattle, bovine spongiform encephalopathy, immunoblotting, brain, diagnostic techniques and value, histopathology, electron microscopy, prion proteins, UK.

67. Corn, Joseph L.; Nettles, Victor F. Health protocol for translocation of free-ranging elk. Journal of Wildlife Diseases 37(3), July, 2001: 413-426.
    NAL call no. 41.9 W64B
    Descriptors: elk, Cervus elaphus, health protocol for restoration programs, health scoring, ectoparasites, some diseases, internal parasites, quarantines, diagnostic testing, prophylactic treatment.

68. Coulthart, M.B.; Cashman, N.R. Variant Creutzfeldt-Jakob disease: a summary of current scientific knowledge in relation to public health. CMAJ-Canadian Medical Association Journal 2001 Jul 10; 165(1): 51-58. ISSN: 0820-3946
    Abstract: The prion diseases pose unique scientific, medical, veterinary and regulatory challenges. Here, we summarize current information bearing on the natural history, pathobiology and epidemiology of these disorders and public policy responses to the potential threats to public health posed, particularly, by bovine spongiform encephalopathy and variant Creutzfeldt-Jakob disease (vCJD). Six years after the first case reports of vCJD, there is still no clear indication of the magnitude of the primary epidemic, or of the likelihood of lateral transmission of this untreatable disease by iatrogenic means, particularly by blood and blood products. However, the unsettling nature of the available evidence warrants prudence regarding public health policy and regulation, as well as a forward-looking approach to research.
    NAL call no. R11 C3
    Descriptors: NvCJD, natural history, pathobiology, epidemiology, public health risks, UK, transmission issues, iatrogenic transfer.

69. Cousens, S.; Smith, P.G.; Ward, H.; Everington, D.; Knight, R.S.; Zeidler, M.; Stewart, G.; Smith-Bathgate, E.A.; Macleod, M. A.; Mackenzie, J.; Will, R.G. Geographical distribution of variant Creutzfeldt-Jakob disease in Great Britain, 1994-2000. Lancet. 2001 Mar 31. 357(9261): 1002-1007 ISSN: 0140-6736
    Abstract: BACKGROUND: Geographical variation in the distribution of variant Creutzfeldt-Jakob disease (vCJD) might indicate the transmission route of the infectious agent to man. We investigated whether regional incidences of vCJD were correlated with regional dietary data. METHODS: The National CJD Surveillance Unit prospectively identified 84 people with vCJD up to Nov 10, 2000, in Great Britain. Their lifetime residential histories were obtained by interviews with a close relative. Cumulative incidences of vCJD by standard region were calculated. Grid references for places of residence in 1991 were identified and evidence of geographical clusters were sought. Data on diet in the 1980s were analysed for regional correlations with vCJD incidence. The socioeconomic status of the places of residence of people with vCJD was compared with that of the general population. FINDINGS: vCJD incidence was higher in the north of Great Britain than the south. The rate ratio (north vs south) was 1.94 (95% CI 1.27-2.98). The mean Carstairs' deprivation score for areas of residence of people with vCJD was -0.09 (-0.73 to 0.55), which is close to the national average of zero. Regional rates of vCJD were correlated with consumption of other meat or meat products as classified and recorded by the Household Food Consumption and Expenditure Survey (r=0.72), but not with data from the Dietary and Nutritional Survey of British Adults. Five people with vCJD in Leicestershire formed a cluster (p=0.004). INTERPRETATION: Regional differences in vCJD incidence are unlikely to be due to ascertainment bias. We had difficulty determining whether regional variations in diet might cause these differences, since the results of dietary analyses were inconsistent.
    NAL call no. 448.8 L22
    Descriptors: disease incidence, NvCreutzfeldt-Jakob Disease, diet analysis, socioeconomic levels, regional differences, Carstairs’ deprivation score, results.

70. Crise de l'ESB: le secteur bovin toujours dans l'incertitude. [The BSE crisis: still uncertainty for the cattle sector.] Chambres-d'Agriculture. 2001, No. 896, 3-9. ISSN: 0396-7883. In French.
    NAL call no. 14 T69
    Descriptors: BSE, effects on beef consumption and international trade, bovine spongiform encephalopathy, cattle diseases, 2 scenarios, cattle prices, prion diseases, GATT trade agreements

71. Cutlip, R.C.; Miller, J.M.; Hamir, A.N.; Peters, J.; Robinson, M.M.; Jenny, A.L.; Lehmkuhl, H.D.; Taylor, W.D.; Bisplinghoff, F.D. Resistance of cattle to scrapie by the oral route. Canadian Journal of Veterinary Research; Revue Canadienne de Recherche Veterinaire. 2001 Apr. 65(2): 131-132 ISSN: 0830-9000
    Abstract: Early epidemiological information indicated that bovine spongiform encephalopathy (BSE) originated from scrapie in sheep. The question arose if scrapie in North America would induce a BSE-like disease in cattle. Six years ago, we reported that brain tissue from sheep with scrapie caused a neurologic disease when injected directly into the brains of cattle, but the disease induced was different from BSE as it occurs in the United Kingdom and Europe. Here, we report that cattle fed raw brain or meat and bone meal and tallow prepared from sheep with scrapie remained normal for 8 years after exposure. This work indicates that cattle are highly resistant to North American scrapie by the oral route.
    NAL call no. SF601.C24
    Descriptors: BSE, scrapie, cattle, sheep, North American scrapie, oral feeding of raw brain and meat, resistance to infection.

72. Cyranoski, D. Japan's first BSE case fuels fears elsewhere. Nature. 2001 Sep 27. 413(6854): 337 ISSN: 0028-0836
    NAL call no. 472 N21
    Descriptors: BSE, Japan, cattle, introduced zoonosis.

73. de Vries, H. Het afvoeren van alle dieren van een bedrijf naar de destructie bij het aantonen van BSE van een dier op dat bedrijf. Een goede strategie? [Destruction of all animals on the farm after diagnosis of BSE in one animal. Is that a good strategy?] Tijdschrift voor Diergeneeskunde 2001 Feb 15. 126(4): 113-115 ISSN: 0040-7453. In Dutch.
    NAL call no. 41.8 T431
    Descriptors: BSE, bovine spongiform encephalopathies, cattle, livestock, disease control, veterinary care issues, The Netherlands.

74. Dealler, S. Should young UK cattle be considered free of BSE or is it endemic? British Food Journal. 2001, 103: 4, 264-280; 30 ref. ISSN: 0007-070X
    NAL call no. 389.8 B77
    Descriptors: BSE, bovine spongiform encephalopathy; calves; disease transmission; epidemiology; law; pathogenesis; reviews

75. Debeer, S.O.; Baron, T.G. Bensik, A.A. Immunohistochemistry of PrPsc within bovine spongiform encephalopathy brain samples with graded autolysis. Journal of Histochemistry and Cytochemistry 2001 Dec. 49(12): 1519-1524 ISSN: 0022-1554
    Abstract: Bovine spongiform encephalopathy (BSE) is a transmissible neurodegenerative disease of cattle. Clinical diagnosis can be confirmed by investigation of both spongiform changes and abnormal prion protein (PrPsc), a marker considered specific for the disease. Tissue autolysis, often unavoidable in routine field cases, is not compatible with histological examination of the brain even though PrPsc is still detectable by immunoblotting. To determine how autolysis might affect accurate diagnosis using PrPsc immunohistochemistry, we studied 50 field samples of BSE brainstem (obex) with various degrees of autolysis. We demonstrated that the antigen-unmasking pretreatments necessary for PrPsc immunohistochemistry were compatible with the preservation of autolyzed brain sections and that PrPsc detection was unaffected by autolysis, even though anatomic markers were sometimes lost. In tissue samples in which anatomic sites were still recognizable, PrPsc accumulation was detected in specific gray matter nuclei. In samples with advanced autolysis, PrPsc deposits were still observed, at least at the cellular level, as an intraneuronal pattern. We found that the sensitivity of PrPsc immunohistochemistry as a diagnostic method for BSE was undiminished even by severe tissue autolysis.
    NAL call no. 381 J8222
    Descriptors: BSE, diagnosis, brainstem tissue, field samples, method sensitivity.

76. Deslys, J.P.; Lasmezas, C.I.; Comoy, E.; Domont, D. Diagnosis of bovine spongiform encephalopathy. Vet J. London : Balliere Tindall, c1997-. Jan 2001. v. 161 (1) p. 1-3. ISSN: 1090-0233
    NAL call no. SF601.V484
    Descriptors: bovine spongiform encephalopathy, diagnosis, diagnostic techniques.

77. Deslys, Jean Philippe.; Picot, Andre. La vache folle: les risques pour l'homme. [Mad cow disease: the risks for humans.] Dominos (Paris, France); 229. Paris : Flammarion, c2001. 127 p., ill., map. In French. ISBN: 2080300261
    NAL call no. SF967.B63D47 2001
    Descriptors: bovine spongiform encephalopathy, public health risk assessment.

78. Deslys, J. P.; Comoy, E.; Hawkins, S.; Simon, S.; Schimmel, H.; Wells, G.; Grassi, J.; Moynagh, J. Screening slaughtered cattle for BSE. Nature. 2001 Jan 25. 409(6819): 476-478 ISSN: 0028-0836
    NAL call no. 472 N21
    Descriptors: brain, International organizations, slaughter survey, abattoirs, prion protein, bovine spongiform encephalopathy; European Union, cattle.

79. Doherr, M.G.; Heim, D.; Fatzer, R.; Cohen, C.H.; Vandevelde, M.; Zurbriggen, A. Targeted screening of high-risk cattle populations for BSE to augment mandatory reporting of clinical suspects. Prev. Vet. Med. Amsterdam, The Netherlands : Elsevier Science B.V. Sept 20, 2001. v. 51 (1/2) p. 3-16. ISSN: 0167-5877. In the special issue: Structure mirrors function / edited by M. Thrusfield and E. Goodall. Paper presented at a meeting held March 29-31, 2000, Edinburgh, Scotland, UK.
    Abstract: In Switzerland, the first case of bovine spongiform encephalopathy (BSE) was diagnosed in November 1990. Case numbers peaked in 1995, with a total of 352 BSE cases identified by 30 April 2000. Reporting of clinically suspect cattle is currently the most commonly used method world-wide to detect BSE cases. The effectiveness of mandatory reporting depends on a variety of factors; for other diseases passive surveillance underestimates the incidence of clinical cases. The efficiency of passive surveillance systems for BSE will remain unknown until screening tests able to identify clinically affected cattle have been applied in several countries. This paper provides the first detailed description of a targeted screening programme for BSE. Two populations of cows >24 months of age were included in the targeted screening: (i) cows found dead or culled on site where the carcass was submitted to rendering (fallen stock) and (ii) cows with health-related problems unfit for routine slaughter that were slaughtered under emergency procedures (emergency slaughter). Between 1992 and 1999, on average 81 clinical BSE suspects per year were reported to the veterinary authorities (passive surveillance), of which 43% were confirmed with BSE. A total of 30 clinical cases were captured by passive surveillance and an additional 20 BSE cases detected by targeted screening between May 1999 and April 2000. The odds of finding a BSE case was 49 times higher in the fallen stock and 58 times higher in emergency-slaughtered cattle when compared to passive surveillance. The targeted screening of fallen stock and emergency-slaughtered cattle considerably increased the number of detected cases in this 12-month period. Targeted-screening cases were on average 4 months younger than the clinical suspect cases. In conclusion, post-mortem testing of fallen stock and emergency-slaughtered cows >24 months for BSE is an important active surveillance element within a total surveillance system that principally is based on mandatory reporting of clinical suspect cases. Without ante-mortem screening tests to detect BSE-infected cattle during the incubation period, a combination of effectively functioning passive and active BSE surveillance strategies might be the only approach to assess the BSE situation reliably in a given country or region - and it is necessary to substantiate claims of freedom from the disease.
    NAL call no. SF601.P7
    Descriptors: cattle, bovine spongiform encephalopathy, screening, monitoring, diagnosis, identification, outbreaks, incidence, detection, efficacy, postmortem examinations, mortality, epidemiology, Switzerland.

80. Dominguez-Carmona, M. Epidemiologia de las enfermedades prionicas animales. [Epidemiology of the animal prion diseases] Anales de la Real Academia Nacional de Medicina (Madr.). 2001. 118(1): 233-245; discussion 245-258. ISSN: 0034-0634 In Spanish.
    Abstract: The authors review the epidemiology of spongiform encephalitis, discussing the possible mechanism of appearance of the epidemic bovine spongiform encephalitis epidemic, leaving aside to another publication the epidemiology of the human spongiform encephalitis and its pathogeny.
    Descriptors: review, epidemiology, BSE, spongiform encephalopathies.

81. Dormont, D. Les mecanismes de la mort neuronale. [Mechanisms of neuron death.] Biofutur. 2001, No. Hors Serie, 22-25; 7 ref. ISSN: 0294-3506. In French.
    NAL call no. TP248.13 B565
    Descriptors: BSE, apoptosis, bovine spongiform encephalopathy, central nervous system lesions, cytokines, free radicals, human diseases, neurons, pathogenesis, pathology, prion peptide 106-126, prion diseases.

82. Dove, A. US throws money at TSE research. Nature Medicine. 2001 Oct. 7(10): 1075 ISSN: 1078-8956
    Descriptors: US, transmissible spongiform encephalopathies, prion diseases, research funding initiatives.

83. Dumas, E., Cabre, O., Bornert, G. Mesures prises en France pour la prevention de la transmission de l'Encephalopathie Spongiforme Bovine. [French mandatory regulations to prevent transmission of Bovine Spongiform Encephalopathy.] Medecine et Armees. Juillet, 2001; 29 (4): 363-368. In French. ISSN: 0300-4937
    Descriptors: Bovine Spongiform Encephalopathy, BSE, food safety concern, mandatory regulations, prevention strategies, surveillance, use of animal proteins in animal feeds, screening tests.

84. Enders, M.; Frohlich, E.; Hassler, D.; Kretzschmar, H. BSE und die neue Variante der Creutzfeldt-Jakob-Krankheit. [BSE (bovine spongiform encephalopathy) and the new variant of Creutzfeldt-Jakob disease] Deutsche medizinische Wochenschrift 2001 Jan 26. 126(4): A55-A56 ISSN: 0012-0472
    NAL call no. 448.8 D48
    Descriptors: NvCreutzfeldt-Jakob Disease, BSE, relationship, human health risks.

85. Engvall, A.; Elvander, M. Riskvardering av lander avseende BSE. [Risk assessment of countries with regard to BSE.] Svensk Veterinartidning. 2001, 53: 8-9, 451-454; 4 ref. ISSN: 0346-2250 In Swedish.
    NAL call no. 41.9 SV23
    Descriptors: BSE, bovine spongiform encephalopathy, European Union, geographical distribution, human health risk assessment.

86. Enserink, M. Infectious diseases. Is the U.S. doing enough to prevent mad cow disease? Science. 2001 Jun 1. 292(5522): 1639-1641 ISSN: 0036-8075
    NAL call no. 470 Sci2
    Descriptors: BSE, scrapie, destruction of Vermont heard of sheep, prevention safeguards, border controls, prion diseases, chronic wasting disease in deer and elk, transmissible mink encephalopathy, testing measures, feed contamination, risk of bovine-based pharmaceuticals.

87. Enserink, M. Prion diseases. U.S. gets tough against chronic wasting disease. Science. 2001 Nov 2. 294(5544): 978-979 ISSN: 0036-8075
    NAL call no. 470 Sci2
    Descriptors: Deer, disease outbreaks, prion disease, wasting syndrome, animal husbandry, chronic disease, prevention and control.

88. Eskens, U. Bovine spongiform encephalopathy (BSE)/transmissible spongiform encephalopathy/mad cow disease. Environmental Science and Pollution Research International 2001. 8(2): 79-83 ISSN: 0944-1344
    Descriptors: BSE, issues, public health concerns, pollution concerns.

89. Estades, J.; Barbier, M.; Remy, E.; Aubert, F. (ed.); Sylvestre, J.P. TI: Le comite d'experts comme dispositif de production de confiance dans la gestion publique des risques: le cas de l'ESB. [The committee of experts as the mechanism for the production of confidence in the management of public risks: the case of BSE.] Confiance et rationalite, Dijon,-France, 5-6-mai 1999. 2001, 113-129; 31 ref. ISBN: 2-7380-0963-8. Publisher: Institut National de la Recherche Agronomique; Paris; France. In French.
    Descriptors: bovine spongiform encephalopathy, cattle diseases, food marketing, food safety, health, meat products, prion diseases, public opinion, public relations

90. FDA finds more evidence of BSE compliance problems. Journal of the American Veterinary Medical Assoc. 2001 Aug 15. 219(4): 427. ISSN: 0003-1488
    NAL call no. 41.8 Am3
    Descriptors: BSE, US feed industry, compliance in using animal based products, ruminant feeds.

91. Federici, C. La reazione del mercato alla vacca pazza. [Reaction of the Italian meat market to mad cow disease.] Rivista di Avicoltura. 2001, 70: 2, 8-10. ISSN: 0005-2213. In Italian.
    NAL call no. 47.8 R523
    Descriptors: beef consumption decline, BSE, bovine spongiform encephalopathy; pork and poultry meat increases, trends.

92. Ferguson-Smith, M.A. BSE and variant CJD. Assumption that BSE originated from scrapie in sheep led to misjudgment. BMJ Clinical Research ed. 2001 Jun 23. 322(7301): 1544-1545. ISSN: 0959-8138
    Descriptors: bovine spongiform encephalopathy, NvCreutzfeldt-Jakob Disease, scrapie, etiology, theories.

93. Foster, J. D.; Parnham, D.; Chong, A.; Goldmann, W.; Hunter, N. Clinical signs, histopathology and genetics of experimental transmission of BSE and natural scrapie to sheep and goats. The Veterinary Record 2001 Feb 10. 148(6): 165-171 ISSN: 0042-4900.
    Abstract: This paper compares the clinical signs, histopathology, detection of PrPSc protein and PrP genetics of the transmission of BSE to sheep and goats, with the effects of the transmission of natural scrapie from a brain homogenate from a single sheep. After intracerebral and oral inoculations there were similarities in the clinical signs due to the two sources of infection, but there were differences in pathology at the end stage of disease and in the genotypes of the sheep which succumbed to the challenges. The incubation period of BSE was associated with the sheep PrP codon 171 genotype, but the natural scrapie source, despite inducing disease only in known susceptible genotypes, showed no clear association with PrP genotype.
    NAL call no. 41.8 V641
    Descriptors: transmissibility, prion disease, scrapie, bovine spongiform encephalopathy, sheep, goats, histopathology, comparison study, PrP Sc, incubation period, behavior, genotype effects.

94. Foster, J. D.; Parnham, D.W.; Hunter, N.; Bruce, M. Distribution of the prion protein in sheep terminally affected with BSE following experimental oral transmission. Journal of General Virology. 2001 Oct. 82(Pt 10): 2319-2326 ISSN: 0022-1317
    Abstract: This study has examined the distribution of PrP(Sc) in sheep by immunocytochemistry of tissues recovered from terminally affected animals following their experimental infection by the oral route with BSE. Despite a wide range of incubation period lengths, affected sheep showed a similar distribution of high levels of PrP(Sc) throughout the central nervous system. PrP(Sc) was also found in the lymphoid system, including parts of the digestive tract, and some components of the peripheral nervous system. These abundant PrP(Sc) deposits in sheep in regions outside the central nervous system are in direct contrast with cattle infected with BSE, which show barely detectable levels of PrP(Sc) in peripheral tissues. A number of genetically susceptible, challenged animals appear to have survived.
    NAL call no. QR360.A1J6
    Descriptors: prion protein, PrP(Sc), immunocytochemistry, oral experimental infections, levels in lymphoid and nervous tissue.

95. Foster, J.; Goldmann, W.; Parnham, D.; Chong, A.; Hunter, N. Partial dissociation of PrPSc deposition and vacuolation in the brains of scrapie and BSE experimentally affected goats. J Gen Virol. Reading : Society for General Microbiology. Jan 2001. v. 82 (pt.1) p. 267-273. ISSN: 0022-1317
    Abstract: The diagnosis of transmissible spongiform encephalopathies (TSEs) depends on the detection of vacuolation in brain sections taken from affected individuals and/or the identification of the disease-associated isoform of the PrP (prion) protein (PrP(Sc)). During the course of an investigation, goats clinically affected following experimental infection with three different sources of TSE (SSBP/1, CH1641 and BSE) developed widespread vacuolar degeneration in the brain. With BSE, PrP(Sc) was clearly recognized in affected goat brain by immunocytochemistry (icc) and Western blotting, but in contrast the experimental scrapie sources SSBP/1 and CH1641 showed almost no or very little PrP(Sc) by icc. Western blot analysis of PrP(Sc) from BSE-affected and SSBP/1-affected goat brain showed that the protein was present in brain affected by both TSE sources, but could not be used to determine how much protein was present. It became clear that PrP(Sc) and vacuolation could be partially dissociated following challenge with two of the three TSE sources. Subtle differences in glycosylation patterns between BSE- and SSBP/1-associated PrP protein isoforms could also be recognized, although these experimentally generated results should not be regarded as a BSE/scrapie differential test. However, our study warns that the reliance on PrP(Sc) determination by icc alone as a means by which to diagnose TSE infection may generate false negative results.
    NAL call no. QR360.A1J6
    Descriptors: bovine spongiform encephalopathy, prion proteins, PrPSc, scrapie, goats, brain changes, diagnostic testing, reliability.

96. Fournier, J. G. Nonneuronal cellular prion protein. International Review of Cytology 2001. 208: 121-160 ISSN: 0074-7696
    Abstract: The normal cellular prion protein (PrP(c)) is a membrane sialoglycoprotein of unknown function having the unique property of adopting an abnormal tertiary conformation. The pathological conformer PrP(sc) would be the agent of transmissible spongiform encephalopathies or prion diseases. They include scrapie and bovine spongiform encephalopathy in animals and Creutzfeldt-Jakob disease in humans. The conversion of PrP(c) into PrP(sc) in the brain governs the clinical phenotype of the disease. However, the three-dimensional structure change of PrP(c) can also take place outside the central nervous system, in nonneuronal cells particularly of lymphoid tissue where the agent replicates. In natural infection, PrP(c) in nonneuronal cells of peripheral extracerebral organs may play a key role as the receptor required to enable the entry of the infectious agent into the host. In the present review we have undertaken a first evaluation of compelling data concerning the PrP(c)-expressing cells of nonneuronal origin present in cerebral and extracerebral tissues. The analysis of tissue, cellular, and subcellular localization of PrP(c) may help us better understand the biological function of PrP(c) and provide some information on physiopathological processes underlying prion diseases.
    NAL call no. 442.8 In82
    Descriptors: prion protein, nonneuronal cerebral and extracerebral organ receptors, physiopathological processes, prion diseases.

97. Fricker, J. BSE crisis--transmission through blood transfusions? Trends in Molecular Medicine. 2001 Jan. 7(1): 2-3 ISSN: 1471-4914
    Descriptors: BSE, risk factors, bovine-based pharmaceuticals, contamination of equipment.

98. Gabus, C.; Auxilien, S.; Pechoux, C.; Dormont, D.; Swietnicki, W.; Morillas, M.; Surewicz, W.; Nandi, P.; Darlix, J. The Prion Protein has DNA Strand Transfer Properties Similar to Retroviral Nucleocapsid Protein. Journal of Molecular Biology 2001 vol. 307, no. 4, pp. 1011-1021 ISSN: 0022-2836
    NAL call no. 442.8 J8224
    Descriptors: transmissible spongiform encephalopathies, PrP, nucleic acids, experimental infection of mice, PrP involvement in nucleic acid metabolism.

99. Gale, P. Developments on microbiological risk assessment for drinking water. J Appl Microbiol. Oxford, U.K. : Blackwell Science Ltd. Aug 2001. v. 91 (2) p. 191-205. ISSN: 1364-5072
    Abstract: This paper considers the development of microbiological risk assessment models for pathogenic agents in drinking water with particular reference to Cryptosporidium parvum, rotavirus and bovine spongiform encephalopathy (BSE). The available evidence suggests that there is potential for considerable variation in exposures to C. parvum oocysts through drinking water, during both outbreak and non-outbreak conditions. This spatial/temporal heterogeneity arises both from variation in oocyst densities in the raw water and fluctuations in the removal efficiencies of drinking water treatment. In terms of risk prediction, modeling the variation in doses ingested by individual drinking water consumers is not important if the dose-response curve is linear and the oocysts act independently during infection. Indeed, the total pathogen loading on the population as represented by the arithmetic mean exposure is sufficient for risk prediction for C. parvum, BSE and other agents of low infectivity, providing the infecting particles (i.e. oocysts or BSE prions) are known to act independently. However, for more highly infectious agents, such as rotavirus, ignoring the variation and just using the arithmetic mean exposure may over-estimate the risk by a factor of about threefold. If it were to be shown that pathogens co-operate with each other during initiation of infection, such that the dose-response relationship is non-linear, then modelling the variation in doses ingested by individual consumers would be very important. Possible mechanisms for co-operation of pathogens during infection are considered. Simulations show that acquired protective immunity for C. parvum reduces the risk of infection during outbreak conditions by over 10-fold. Variation in virulence between strains of C. parvum is a further source of uncertainty.
    NAL call no. QR1.J687
    Descriptors: pathogens, water-microbiology, risks of BSE contamination.

100. Gale, P.; Stanfield, G. Towards a quantitative risk assessment for BSE in sewage sludge. Journal of Applied Microbiology. September, 2001; 91 (3): 563-569. ISSN: 1364-5072
     NAL call no. QR1 J687
     Descriptors: Source-Pathway-Receptor approach, BSE, contaminated waste, arithmetic mean concentration of BSE agent, slaughter house effluent, horizontal transmission risk.

101. Garcia, B.M. Encefalopatia espongiforme bovina. [Bovine spongiform encephalopathy.] Alimentaria. 2001, No. Extr., 24-62; 32 ref. ISSN: 0300-5755. In Spanish.
     Descriptors: BSE, bovine spongiform encephalopathy, cattle diseases, Creutzfeldt-Jakob disease, human diseases, nervous system diseases, prion diseases, reviews.

102. Gatnau R.; Polo J.; Robert, E.; Brufau J. Plasma protein antimicrobial substitution at negligible risk. Feed manufacturing in the Mediterranean region. Improving safety: from feed to food. Proceedings of the III Conference of Feed Manufacturers of the Mediterranean, organized by ASFAC, with the collaboration of IRTA, CESFAC, CIHEAM, Patronat Catala Pro Europa and Fira de Reus, and sponsored by Port de Tarragona, Reus (Spain), 22-24 March 2000. Cahiers Options Mediterraneennes. 2001, 54: 141-150; 45 ref. ISSN: 1022-1379. In French with an English summary.
     Descriptors: blood derived proteins, feed ingredients, disease risks, BSE, dioxin, biological safety, correlating blood derived products and health benefits, risk assessment.

103. Gavier Widen, D.; Wells, G.A.; Simmons, M. M.; Wilesmith, J.W.; Ryan, J. Histological observations on the brains of symptomless 7-year-old cattle. Journal of Comparative Pathology. 2001 Jan. 124(1): 52-59 ISSN: 0021-9975
     Abstract: The histological changes in the brains of 506 clinically normal 7-year-old cattle, which were part of a cohort study on maternal transmission of bovine spongiform encephalopathy, are described. Vacuolation of the white matter, of unknown aetiology, located particularly in the substantia nigra, was a frequent finding. Vacuolated neurons were commonly observed in the red nucleus (64.3% of the animals) and in the habenular nucleus (50.1%). Spheroids were found in 10.8% of the brains, most frequently in the vestibular nuclei. Cellular inflammatory infiltrates in association with blood vessels occurred in 30% of the animals at various locations in the brain; their aetiology remains uncertain, but they may have reflected subclinical or latent infections. Mineralization of the wall of blood vessels, with proliferation of the intima, was observed frequently in vessels of the internal capsule and was probably associated with ageing. The description of histological findings in the brain of symptomless adult cattle in the present study provides a useful background for diagnostic bovine neuropathology.
     NAL call no. 41.8 J82
     Descriptors: histological changes, normal 7 year old cattle, maternal BSE transmission, vacuolated neurons, substantia nigra.

104. Gee, R.W. Communication of risk. Australian Veterinary Journal. 2001 Feb. 79(2): 136. ISSN: 0005-0423
     NAL call no. 41.8 Au72
     Descriptors: zoonotic diseases, TSE, media and governmental communications, BSE, UK, Australia.

105. Germany. Gesellschaft fuer Tierzuchtwissenschaft Position statement of the Gesellschaft fuer Tierzuchtwissenschaft (GfT) (Society for Animal Husbandry Science) and the Deutsche Gesellschaft fuer Zuechtungskunde (DGfZ) (German Society for Animal husbandry) regarding the BSE crisis (Bovine Spongiform Encephalitis). Zuechtungskunde. Marz-April, 2001; 73 (2): 81-84. ISSN: 0044-5401. In German.
     NAL call no. 49 Z8
     Descriptors: BSE, Germany societies position paper.

106. Giles, J. Mad cow disease comes to Japan. Nature. 2001 Sep 20. 413(6853): 240 ISSN: 0028-0836
     NAL call no. 472 N21
     Descriptors: cattle, BSE, confirmed case, imported feed, contaminated feed, UK, Japan.

107. Glatzel, Markus; Aguzzi, Adriano. The shifting biology of prions. Brain Research Reviews. October, 2001; 36 (2-3): 241-248. ISSN: 0165-0173.
     Descriptors: BSE, transmissible spongiform encephalopathies, prion and prion diseases.

108. Goldwater, P.N. Bovine spongiform encephalopathy and variant Creutzfeldt-Jakob disease: implications for Australia. Medical Journal of Australia, 2001 Aug 6. 175(3): 154-158 ISSN: 0025-729X
     Abstract: The bovine spongiform encephalopathy (BSE) epizootic developed in the United Kingdom in the mid-1980s. Feeding practices in the cattle industry amplified the causative prion, and meat contaminated with BSE entered the market. Human consumption of prion-contaminated meat led to the new zoonosis--variant Creutzfeldt-Jakob disease (vCJD). The UK BSE Inquiry published its report in October 2000; while praising policy decisions, it also documented failures in the execution of these policies, specifically delays and lack of rigour. Australia is in an excellent position to maintain its BSE- and scrapie-free status, but widespread active surveillance of neural and non-neural tissue from all species of farmed quadrupeds is needed.
     Descriptors: disease free status, BSE, scrapie, Australia.

109. Golsteyn, R. M. When being right is not enough. Trends in Cell Biology 2001 Feb. 11(2): 59 ISSN: 0962-8924
     NAL call no. QH573.T73
     Descriptors: Bovine spongiform encephalopathy, communication, transmission, politics, cattle.

110. Grant, H. BSE and variant CJD. Humans can live with BSE so long as they do not eat brains. BMJ-Clinical Research ed. 2001 Jun 23. 322(7301): 1545. ISSN: 0959-8138
     Descriptors: bovine spongiform encephalopathy, NvCreutzfeldt-Jakob Disease, scrapie, etiology, theories, diet, human health risks.

111. Gravenor, M. B.; Cox, D. R.; Hoinville, L. J.; Hoek, A.; McLean, A.R. The flock-to-flock force of infection for scrapie in Britain. Proceedings of the Royal Society of London. Series B. Biological Sciences. 2001 Mar 22. 268(1467): 587-592 ISSN: 0962-8452
     Abstract: A postal survey of British sheep farmers provided information on the proportion of farms that experienced their first case of scrapie in each year between 1962 and 1998. We found no evidence of a large increase in the proportion of scrapie-affected farms prior to, during or following the epidemic of BSE in British cattle. After correcting for between-farm heterogeneity in the probability of acquiring scrapie, we estimated the yearly between-flock force of infection since 1962. The current force of infection is estimated at approximately 0.0045 per farm per year and combined with a simple model of scrapie spread provides an estimate of the average duration of a scrapie outbreak on an individual farm. Considering all farms, the average outbreak lasts for five years, but if only those farms that have cases in animals born on the farm are considered, it lasts 15 years. We use these parameter estimates to compare the proportion of farms with scrapie in time periods of different lengths. In the survey, 2.7% of farms had a case in 1998. The 5.3% of farms reporting having a case between 1993 and 1997 is consistent with the hypothesis that the scrapie force of infection remained constant over this period.
     NAL call no. 501 L84B
     Descriptors; Disease outbreaks, scrapie transmission, epidemiology, prevention and control, sheep, diseases, cattle, bovine spongiform encephalopathy, Great Britain, statistical models, questionnaires.

112. Griffiths, P.D. Variant CJD epidemiology: joining up the dots. Reviews in Medical Virology 2001 vol. 11, no. 4, pp. 203-204 ISSN: 1052-9276.
     Descriptors: BSE, disease transmission, NvCreutzfeldt-Jakob-disease, incidence of disease, mathematical model of human exposure, disease projection.

113. Gross, John E.; Miller, Michael W. Chronic wasting disease in mule deer: Disease dynamics and control. Journal of Wildlife Management. Bethesda, MD: The Wildlife Society. April 2001 v. 65 (2) p. 205-215. ISSN: 0022-541X
     Abstract: The authors developed a mechanistic model to simulate the dynamics of CWD in mule deer populations. Estimated parameters were projected for age-specific disease dynamics, changes in population size and effects of control strategies. The parameters were estimated from observations of infections and uninfected deer in Colorado. They found that the culling rate (20% of infected populations) effectively eliminated the disease at low disease levels. When disease rates were high, the likelihood of disease control diminished rapidly. To eliminate CWD from wild populations may take decades.
     NAL call no. 41.9 W64B
     Descriptors: chronic wasting disease, (CWD) transmissible spongiform encephalopathies, Odocoileus hemionus, mule deer, PrPres, prion protein, epidemiology, game-farmed wapiti, disease control measure, wildlife populations, wildlife management, public health threats, mathematical model, Monte Carlo techniques

114. Gunn, M. Observations on disposal of BSE-infected carcasses. Irish Veterinary Journal. 2001, 54: 4, 192-193; 4 ref. ISSN: 0368-0762
     NAL call no. 41.8 IR4
     Descriptors: BSE, bovine spongiform encephalopathy, brain, carcass disposal, sources of infection, human risk assessment.

115. Guo, Zhi Ru; Jin, NingYi; Guo, Z.R.; Jin, N.Y. Advances in research on bovine spongiform encephalopathy. Chinese Journal of Veterinary Science. 2001, 21: 3, 307-311; 49 ref. ISSN: 1005-4545. In Chinese.
     NAL call no. SF604.J68
     Descriptors: Bovine spongiform encephalopathy, research, reviews.

116. Half a response. Vet Rec. London : The British Veterinary Association. Feb 17, 2001. v. 148 (7) p. 189. ISSN: 0042-4900
     NAL call no. 41.8 V641
     Descriptors: bovine spongiform encephalopathy, guidelines, UK.

117. Hamir, A.N.; Cutlip, R.C.; Miller, J.M.; Williams, E.S.; Stack, M.J.; Miller, M.W.; O'Rourke, K.I.; Chaplin, M.J. Preliminary findings on the experimental transmission of chronic wasting disease agent of mule deer to cattle. J Vet Diagn Invest. Lawrence, Kan. : AAVLD. Jan 2001. v. 13 (1) p. 91-96. ISSN: 1040-6387
     NAL call no. SF774.J68
     Descriptors: cattle, mule deer, chronic wasting disease, Odocoileus hemionus, spongiform encephalopathy, disease transmission, experimental infections, disease course, brain lesions, diagnostic techniques, prion proteins.

118. Hammer, G.F. Massnahmen-Bundel fur mehr Sicherheit: die tierseuchen- und fleischhygienerechtliche Vorschriften zur BSE-Bekampfung. [Combined measures for more security: the regulations relating to animal epidemics and meat hygiene for BSE control.] Fleischwirtschaft. 2001, 81: 4, 54-60; 25 ref. ISSN: 0015-363X. In German.
     NAL call no. 280.38 F62
     Descriptors: animal health, BSE, bovine spongiform encephalopathy, cattle, meat product safety measures, prion diseases, regulations, Germany.

119. Haunhorst E. Lebensmitteluberwachung: Umsetzung der BSE-Regelungen Massnahmen Massnahmen der Lander sollen Schutz der Verbraucher gewahrleisten. [Implementation of BSE regulations to protect consumers.] Fleischwirtschaft. 2001, 81: 6, 20-22. ISSN: 0015-363X. In German.
     NAL call no. 280.38 F62
     Descriptors: BSE, bovine spongiform encephalopathy; legislation implementation, German provinces.

120. Healy, B. vCJD: broad U.S. response required. Science. 2001 Mar 9. 291(5510): 1859 ISSN: 0036-8075
     NAL call no. 470 Sci2
     Descriptors: Creutzfeldt-Jakob Syndrome, diagnosis, prevention and control, cattle, lymphoid tissue, population surveillance, United States.

121. Heeschen, W.H. Bovine Spongiforme Enzephalopathie (BSE). "Milch ist als sicher anzusehen" -- derzeitiger Stand wissenschaftlicher Erkenntnisse zu dieser Aussage. [Bovine spongiform encephalopathy (BSE). "Milk is safe" -- current state of scientific results regarding this quote.] DMZ, Lebensmittelindustrie und Milchwirtschaft. 2001, 122: 2, 60-67; 17 ref. ISSN: 0938-9369. In German.
     NAL call no. HD9275 G3D59
     Descriptors: milk, food safety, food contamination, human health risk assessment, BSE, bovine spongiform encephalopathy; cattle prion diseases.

122. Heppner, Frank L.; Arrighi, Isabelle; Kalinke Ulrich; Aguzzi, Adriano. Immunity against prions? Trends in Molecular Medicine. November, 2001; 7 (11): 477-479. ISSN: 1471-4914
     Descriptors: prion protein, antibodies, scrapie-infected cells in vitro, feasibility of immunotherapeutical intervention, question effectiveness of vaccines and post-exposure strategies based on antibodies scrapie, BSE, Creutzfeldt-Jakob disease

123. Heppner, F. L.; Musahl, C.; Arrighi, I.; Klein, M. A.; Rulicke, T.; Oesch, B.; Zinkernagel, R. M.; Kalinke, U.; Aguzzi, A. Prevention of scrapie pathogenesis by transgenic expression of anti-prion protein antibodies. Science. 2001 Oct 5. 294(5540): 178-182 ISSN: 0036-8075
     Abstract: Variant Creutzfeldt-Jakob disease and bovine spongiform encephalopathy are initiated by extracerebral exposure to prions. Although prion transmission from extracerebral sites to the brain represents a potential target for prophylaxis, attempts at vaccination have been limited by the poor immunogenicity of prion proteins. To circumvent this, we expressed an anti-prion protein (anti-PrP) mu chain in Prnp(o/o) mice. Transgenic mice developed sustained anti-PrP titers, which were not suppressed by introduction of Prnp+ alleles. Transgene expression prevented pathogenesis of prions introduced by intraperitoneal injection in the spleen and brain. Expression of endogenous PrP (PrP(C)) in the spleen and brain was unaffected, suggesting that immunity was responsible for protection. This indicates the feasibility of immunological inhibition of prion disease in vivo.
     NAL call no. 470 Sci2
     Descriptors: prion diseases, anti-prion protein, mouse model, introperitoneal injection, spleen, brain, immunity response, possibilities for effective vaccine.

124. Hill, A.F.; Collinge, J.; Rabenau, H.F. (ed.); Cinatl, J. (ed.); Doerr, H.W. Strain variations and species barriers. Prions: a challenge for science, medicine and public health-system. 2001, 48-257; 48 ref. Published by: S. Karger AG; Basel; Switzerland. ISBN: 3-8055-7124-0
     NAL call no. QR1 C66 v. 7
     Descriptors: BSE, bovine spongiform encephalopathy, Creutzfeldt Jakob disease, prion diseases, scrapie, strain differences, species barriers.

125. Hof, G. Verwarring rond boviene spongiforme encefalopathie (BSE) en het risico op de nieuwe variant van de ziekte van Creutzfeldt-Jakob. [Confusion surrounding bovine spongiform encephalopathy (BSE) and the risk of new variant Creutzfeldt-Jakob disease] Nederlands Tijdschrift voor Geneeskunde 2001 Mar 10. 145(10): 501-502. ISSN: 0028-2162. In Dutch.
     Descriptors: BSE, transmissible spongiform encephalopathies, NvCreutzfeldt-Jakob disease, human health risks, exposure to contaminated bovine-based products.

126. Hossain, H.; Chakraborty, T. Prion-Krankheiten. [Prion diseases] Anasthesiologie, Intensivmedizin, Notfallmedizin, Schmerztherapie-AINS. 2001 Jan. 36(1): 15-24 ISSN: 0939-2661. In German.
     Descriptors: prions, prion diseases, BSE, CJD, human health risks.

127. Huillard-d'Aignaux, J. N.; Cousens, S.N.; Smith, P.G. Predictability of the UK variant Creutzfeldt-Jakob disease epidemic. Science. 2001 Nov 23. 294(5547): 1729-1731 ISSN: 0036-8075
     Abstract: Back-calculation analysis of the variant Creutzfeldt-Jakob disease epidemic in the United Kingdom is used to estimate the number of infected individuals and future disease incidence. The model assumes a hazard of infection proportional to the incidence of bovine spongiform encephalopathy in the United Kingdom and accounts for precautionary control measures and very wide ranges of incubation periods. The model indicates that current case data are compatible with numbers of infections ranging from a few hundred to several millions. In the latter case, the model suggests that the mean incubation period must be well beyond the human life-span, resulting in disease epidemics of at most several thousand cases.
     NAL call no. 470 Sci2
     Descriptors: BSE, back-circulation analysis model, epidemiology, human health risks, disease incidence projection

128. Investigation of vCJD cluster points to butchering practices. Clinical Infectious Diseases 2001 Jun 15. 32(12): ii. ISSN: 1058-4838
     NAL call no. RC111 R4
     Descriptors: cattle slaughter processes, BSE, NvCreutzfeldt-Jakob Disease, incidence, disease transmission risks.

129. Jackson, G.S.; Collinge, J. The molecular pathology of CJD: old and new variants. Mol Pathol. 2001 Dec. 54(6): 393-399 ISSN: 1366-8714
     Abstract: The study of prion disease has become an area of intense interest since experimental evidence emerged for the transmission of phenotypic variation without the involvement of a nucleic acid component. Additional impetus has come from the widespread concern that exposure to bovine spongiform encephalopathy contaminated material poses a distinct and, conceivably, a severe threat to public health in the UK and other countries. The occurrence of new variant Creutzfeldt-Jakob disease has dramatically highlighted the need for a precise understanding of the molecular basis of prion propagation. The molecular basis of prion strain diversity, previously a major challenge to the "protein only" model, can now be reconciled with propagation of infectious protein topologies. The conformational change known to be central to prion propagation, from a predominantly alpha-helical fold to one predominantly comprising beta-structure, can now be reproduced in vitro, and the ability of beta-PrP to form fibrillar aggregates provides a plausible molecular mechanism for prion propagation. Concomitantly, advances in the fundamental biology of prion disease have done much to reinforce the protein only hypothesis of prion replication.
     Descriptors: BSE exposure, public health concerns, NvCreutzfeldt-Jakob Disease, prion proteins, conversion to bets helical structure, in vitro.

130. Jackson, G.S.; Beck, J.A.; Navarrete, C.; Brown, J.; Sutton, P.M.; Contreras, M.; Collinge, J. Pathogenesis: HLA-DQ7 antigen and resistance to variant CJD Nature 2001 vol. 414, no. 6861, pp. 269-270 ISSN: 0028-0836
     NAL call no. 472 N21
     Descriptors: NvCreutzfeldt-Jakob disease, bovine spongiform encephalopathy (BSE)-like prion strain, human leukocyte antigen, differential diagnosis, host susceptibility.

131. Japan's beef scandal. Nature. 2001 Sep 27. 413(6854): 333 ISSN: 0028-0836
     NAL call no. 472 N21
     Descriptors: BSE, bovine spongiform encephalopathy, NvCreutzfeldt-Jakob Disease, biased government communications, Japan, confirmed case, cattle, feed contamination concerns, UK, meat and bone meal imports.

132. Jeffrey M.; Martin, S.; Gonzalez, L.; Ryder, S.J.; Bellworthy, S.J; Jackman, R. Differential diagnosis of infections with the bovine spongiform encephalopathy (BSE) and scrapie agents in sheep. Journal Comparative Pathology. November, 2001; 125 (4): 271-284. ISSN: 0021-9975
     NAL call no. 41.8 J82
     Descriptors: scrapie, bovine spongiform encephalopathy, BSE, NvCreutzfeldt-Jakob disease, transmissible spongiform encephalopathies, prion diseases, sheep, immunohistochemical methods, brain, lymphoreticular system, experimental and natural infections, strain-dependent processing, strain identification.

133. Jeffrey, M.; Ryder, S.; Martin, S.; Hawkins, S.A.C.; Terry, L.; Berthelin-Baker, C.; Bellworthy, S.J. Oral inoculation of sheep with the agent of bovine spongiform encephalopathy (BSE). 1. Onset and distribution of disease-specific PrP accumulation in brain and viscera. J Comp Pathol. London : W.B. Saunders Company Ltd. May 2001. v. 124 (4) p. 280-289. ISSN: 0021-9975
     Abstract: Sixty-three Romney sheep aged 6 months, consisting of three groups (PrP(ARQ/ARQ), PrP(ARQ/ARR), and PrP(ARR/ARR)genotypes) of 21 animals, were infected orally with brain tissue from BSE-infected cattle. Sub-groups of the 21 PrP(ARQ/ARQ) animals were killed, together with uninfected controls 4, 10, 16, 22 or 24-28 (after the development of full clinical disease) months post-inoculation (mpi). One sheep from each of the two groups of four killed at 4 or 10 mpi were shown by immunohistochemical examination to possess disease-specific PrP accumulations in single lymph nodes. At 16 mpi, such accumulations were detected in two of four infected sheep in some viscera and in the spinal cord and brain. At 22 mpi, three of five infected sheep had widespread disease-specific PrP accumulations in all tissues examined, but the remaining two animals gave positive results only in the central nervous system. Clinical disease appeared at 20-28 mpi. Three sheep killed with advanced clinical signs showed widespread PrP accumulation in brain, spinal cord and peripheral tissues. These results confirmed that PrP(ARQ/ARQ) Romney sheep are susceptible to experimental infection with the BSE agent. The different sites at which initial PrP accumulations were detected suggested that the point of entry of infection varied. Once established, however, infection appeared to spread rapidly throughout the lymphoreticular system. The results suggested that in some BSE-infected sheep neuroinvasion occurred in the absence of detectable PrP accumulations in the viscera or peripheral nervous system. In contrast to cattle with BSE, however, most sheep showed disease-specific PrP accumulations in the lymphoreticular system. In this respect, BSE-infected resembled scrapie-infected sheep; it is possible, however, that future research will reveal differences in respect of targeting of cell types within the lymphoreticular and peripheral nervous systems. The PrP(ARQ/ARR)and PrP(ARR/ARR)sheep were also killed in sub-groups at intervals after inoculation. Up to 24 mpi, however, none of these animals showed disease-specific PrP accumulations. Further results will be reported later.
     NAL call no. 41.8 J82
     Descriptors: sheep, bovine spongiform encephalopathy, genotypes, prion proteins, brain experimental infection, spinal cord, animal tissues, clinical aspects, pathogenesis, peripheral nerves.

134. Kaaden, O.R.; Rabenau, H.F. (ed.); Cinatl, J. (ed.); Doerr, H.W. Animal transmissible spongiform encephalopathy: clinical and diagnostic aspects. Prions: a challenge for science, medicine and public health-system. 2001, 145-150; 48 ref. Published by: S. Karger AG; Basel; Switzerland. ISBN: 3-8055-7124-0
     NAL call no. QR1 C66 v. 7
     Descriptors: BSE, bovine spongiform encephalopathy, clinical aspects, diagnosis, pathogenesis, prion diseases, scrapie, animals.

135. Kahrs, R.F. Viral diseases of cattle. 2001, viii + 324 pp. Iowa State University Press; Ames; USA ISBN: 0-8138-2591-1
     NAL call no. SF961 K26 2001
     Descriptors: Aujeszky's disease, bovine leukosis, BSE, bovine spongiform encephalopathy, clinical aspects, disease diagnosis, disinfectants, diagnostic-techniques, FMD, foot and mouth disease, rabies, international trade, malignant catarrhal fever, rhinotracheitis, Rift Valley fever, rinderpest, vaccines, vulvovaginitis, various viral diseases, herpes, warts, poxvirus, etc.

136. Kamphues, J.; Zentek, J.; Oberthur, R.C.; Flachowsky, G.; Coenen, M. Futtermittel tierischer Herkunft als mogliche Verbreitungsursache fur die bovine spongiforme Enzephalopathie (BSE) in Deutschland: 1. Mitteilung: Vergleichende Risikobewertung der Einzelfuttermittel tierischer Herkunft. [Animal-derived feeds as possible vectors for bovine spongiform encephalopathy (BSE) in Germany. 1. Comparative risk assessment for a single animal food of animal origin] Deutsche Tierarztliche Wochenschrift 2001 Jul. 108(7): 283-290. ISSN: 0341-6593. In German.
     Abstract: The occurrence of BSE cases in Germany after the ban of meat and bone meal for ruminant feed in 1994 requires a detailed investigation of animal derived feedstuffs regarding their specific risks as vectors for the disease. Accepting the theory that BSE is a prion transmitted disease, the theoretical infectious potential was calculated for animal derived feedstuffs. This calculation was based on the assumption, that risk material (brain, spinal cord) of one clinically diseased cattle was rendered in the process as established in Germany (133 degrees C, 3 bar, 20 min) or, alternatively, that one diseased animal was slaughtered resulting in normal processing of the by-products for human food production. From this risk assessment it became obvious that meat and bone meal was one, but probably not the most important source for the spreading of BSE. Taking into account the high sensitivity of calves it can be speculated that certain products, e.g. from bone processing (bone meal) and fat melting (mixed animal fats), commonly used for the formulation of milk replacers, might have been more important as pathways. As it can't be excluded retrospectively that infected meat and bone meal was imported from the UK, this non-calculable influence may have been related to the significance of the other products. The calculation model underlines that efficient removal of specified risk material (brain, spinal cord) and adequate processing (133 degrees C, 3 bar, 20 min) or alternatively other equivalent treatments of fats are prerequisites for minimizing the risk of feed borne transmission of BSE by animal derived feedstuffs. The epidemiological consequences are part of a subsequent paper.
     NAL call no. 41.8 D482
     Descriptors: contaminated feeds, meat and bone meal, feed processing, cattle, risk assessment.

137. Kingombe, Cesar Isigidi Bin; Luthi, Elisabeth; Schlosser, Heidi; Howald, Denise; Kuhn, Monika; Jemmi, Thomas. A PCR-based test for species-specific determination of heat treatment conditions of animal meals as an effective prophylactic method for bovine spongiform encephalopathy. Meat Science. January, 2001; 57 (1): 35-41. ISSN: 0309-1740
     NAL call no. TX373 M4
     Descriptors: animal waste sterilization, animal based feeds, ELISA and PCR assays, pork tissue, bovine tissue, screening method.

138. Kleinhanss W.; Haxsen G.; Uhlmann F.; Kamphues J. (ed.); Flachowsky G. Okonomische Bewertung eines Verbots der Tiermehlherstellung und verfutterung. [Economic assessment of a ban on meat and bone meal production and feeding.] Animal nutrition - resources and future developments. Workshop on Sustainable Animal Production, 15-16 June 2000, EXPO 2000, Hannover, Germany. Landbauforschung-Volkenrode,-Sonderheft. 2001, No. 223, 159-166; 1 ref. ISSN: 0376-0723 ISBN: 3-933140-47-1. In German with an English summary.
     Descriptors: economic impact, alternative rendering system, ban on meat and bone meals, waste disposal, carcass disposal, BSE.

139. Klimuszko, D. Priony -- czynnik etiologiczny gabczastych encefalopatii. [Prions in the aetiology of spongiform encephalopathies.] Zycie Weterynaryjne. 2001, 76: 3, 128-130; 14 ref. ISSN: 0137-6810. In Polish.
     NAL call no. SF604.Z9
     Descriptors: BSE, etiology, bovine spongiform encephalopathy, prion diseases, pathogenesis, public health risks, scrapie.

140. Kolb, E. Neuere Erkenntnisse uber die Eigenschaften des Erregers der BSE und der Traberkrankheit sowie uber die Infektionswege (Ubersichtsreferat). [Recent studies on the properties of BSE and scrapie infectious agents and mechanisms of infection.] Tierarztliche Umschau. 2001, 56: 4, 175-183; 37 ref. ISSN: 0049-3864. In German with an English summary.
     NAL call no. 41.8 T445
     Descriptors: BSE, bovine spongiform encephalopathy, prion disease, scrapie, sheep, cattle, disease process.

141. Koo, Hye Cheong; Park, Yong Ho; Lee, Byeong Chun; Chae, Chan Hee; O' Rourke K.I.; Baszler, T.V.; Koo, H.C.; Park Y.H.; Lee, B.C.; Chae, C.H. Immunohistochemical detection of prion protein (PrP-Sc) and epidemiological study of BSE in Korea. Journal of Veterinary Science. 2001, 2: 1, 25-31; 43 ref. ISSN: 1229-845X
     NAL call no. SF604.J68
     Descriptors: BSE, etiology, prion protein, cattle survey, Korea, immunohistochemistry and western immunoblotting assays, epidemiology.

142. Koppinen, J. The French reflect on BSE. Australian Veterinary Journal 2001 Jul. 79(7): 452 ISSN: 0005-0423
     NAL call no. 41.8 Au72
     Descriptors: BSE, the French situation.

143. Kramer, G. N.; Pauwels, J.; Le Guern, L.; Schimmel, H.; Trapmann, S. Recent production of candidate reference materials at IRMM. Fresenius' Journal of Analytical Chemistry. 2001 Jun. 370(2-3): 142-146 ISSN: 0937-0633
     Abstract: In the execution of its mission to promote a common European measurement system in support of EU policies, IRMM's Reference Materials Unit is currently involved in preparation of proficiency-testing samples and candidate reference materials. Recent work related to bovine spongiform encephalopathy in cows, genetically modified organisms, and a variety of environmental materials is described.
     NAL call no. QD71.F7
     Descriptors: EU policies, BSE, measurement systems, reference materials.

144. Krammer, R. BSE crisis sinks German public biotech programme. Nature. 2001 Feb 1. 409(6820): 549 ISSN: 0028-0836
     NAL call no. 472 N21
     Descriptors: bovine spongiform encephalopathy, impacts on research, biotechnology research, Germany.

145. Krcmar, P.; Rencova, E. Identification of bovine-specific DNA in feedstuffs. Journal of food protection 2001 Jan. 64(1): 117-119 ISSN: 0362-028X
     Abstract: Considering the menace of transmission of bovine spongiform encephalopathy, feed components intended for cattle nutrition must be checked for the presence of bovine-derived materials. We have been using a method based on polymerase chain reaction for the identification of bovine-specific mitochondrial DNA sequences for this purpose. The specificity of the primers for polymerase chain reaction has been tested using samples of DNA of other vertebrate species, which may also be present in rendering plant products. The method allows the detection in concentrate mixtures of 0.125% of bovine-derived material. Bovine DNA at concentrations corresponding to less than 0.5% of bovine-derived material was detected in 3 of the 30 samples of concentrate mixtures collected from distributors' stores all over the Czech Republic. All 44 samples of fish meal collected from the same sources were free of bovine-derived material.
     NAL call no. 44.8 J824
     Descriptors: BSE, bovine derived feed testing, feed contamination, mitochondrial DNA testing, polymerase chain reaction method, detection method.

146. Kuzma, C.D. BSE: could it happen here? Experts say probably not. Journal of the American Veterinary Medical Association. 2001 Mar 1. 218(5): 646-647, 650 ISSN: 0003-1488
     NAL call no. 41.8 Am3
     Descriptors: bovine spongiform encephalopathy, cattle, US.

147. Laffling, A.J.; Baird, A.; Birkett, C.R.; John, H.A. A monoclonal antibody that enables specific immunohistological detection of prion protein in bovine spongiform encephalopathy cases. Neuroscience Letters. 2001 Mar 9. 300(2): 99-102 ISSN: 0304-3940
     Abstract: The specificity of a monoclonal antibody (mAB) raised against recombinant bovine prion protein (PrP) for the immunohistological detection of PrP accumulation in the medulla oblongata of bovine spongiform encephalopathy (BSE) and ovine scrapie cases was investigated. mAB KG9 showed a diffuse low intensity reaction with the cytoplasm of neurones in normal cattle and sheep sections. In BSE sections the mAB detected widespread granular deposits of PrP associated with neurones and the neuropil. Although scrapie sections showed similar levels of granular deposits with another antibody to PrP these were not detected by KG9 which did however detect diffuse staining in neuronal cytoplasm. Possible explanations for the specificity of binding of KG9 are discussed.
     NAL call no. QP351 N3
     Descriptors: BSE, diagnosis, cattle, medulla oblongata, ovine scrapie, mAB KG9 antibody, PrP, specificity of KG 9 binding.

148. Lahiff, S.; Glennon, M.; O' Brien, L.; Lyng, J.; Smith, T.; Maher, M.; Shilton, N. Species-specific PCR for the identification of ovine, porcine and chicken species in meat and bone meal (MBM). Molecular and Cellular Probes. February, 2001; 15 (1): 27-35. ISSN: 0890-8508
     NAL call no. RB43.7.M63
     Descriptors: silica-guanidiumthiocyanate DNA isolation procedure, commercial DNA extraction kit, identification of source materials, animal feed formulations, tissue identification, sheep, swine, poultry, transmissible spongiform encephalopathies.

149. Langenfeld, T.W.; Menges, T.; Hempelmann, G. Bovine spongiforme Enzephalopathie (BSE). [Bovine spongiform encephalopathy (BSE)] Anasthesiologie, Intensivmedizin, Notfallmedizin, Schmerztherapie-AINS. 2001 Feb. 36(2): 77-78 ISSN: 0939-2661. In German
     Descriptors: BSE, aspects of the disease, cattle.

150. Lasky, Tamar; Etzel, Ruth; Angulo, Fred; Ward, Hester; Powell, Mark; Rubin, Carol Food safety: Challenges to epidemiology. American Journal of Epidemiology. June 1, 2001; 153 (11 Supplement): S13. ISSN: 0002-9262. Joint Meeting of the Society for Epidemiologic Research, American College of Epidemiology, Epidemiology Section of the American Public Health Association, and the Canadian Society for Epidemiology and Biostatistics, Toronto, Canada, June 13-16, 2001
     NAL call no. 449.8 Am3
     Descriptors: food safety, emerging diseases, public health risks, BSE.

151. Lasmezas, C.I.; Fournier, J.G.; Nouvel, V.; Boe, H.; Marce, D.; Lamoury, F.; Kopp, N.; Hauw, J.J.; Ironside, J.; Bruce, M. Adaptation of the bovine spongiform encephalopathy agent to primates and comparison with Creutzfeldt-Jakob disease: implications for human health. Proc Natl Acad Sci, USA. Washington, D.C. : National Academy of Sciences, Mar 27, 2001. v. 98 (7) p. 4142-4147. ISSN: 0027-8424
     Abstract: There is substantial scientific evidence to support the notion that bovine spongiform encephalopathy (BSE) has contaminated human beings, causing variant Creutzfeldt-Jakob disease (vCJD). This disease has raised concerns about the possibility of an iatrogenic secondary transmission to humans, because the biological properties of the primate-adapted BSE agent are unknown. We show that (i) BSE can be transmitted from primate to primate by intravenous route in 25 months, and (ii) an iatrogenic transmission of vCJD to humans could be readily recognized pathologically, whether it occurs by the central or peripheral route. Strain typing in mice demonstrates that the BSE agent adapts to macaques in the same way as it does to humans and confirms that the BSE agent is responsible for vCJD not only in the United Kingdom but also in France. The agent responsible for French iatrogenic growth hormone-linked CJD taken as a control is very different from vCJD but is similar to that found in one case of sporadic CJD and one sheep scrapie isolate. These data will be key in identifying the origin of human cases of prion disease, including accidental vCJD transmission, and could provide bases for vCJD risk assessment.
     NAL call no. 500 N21P
     Descriptors: bovine spongiform encephalopathy, prions, intravenous injection, disease transmission, Macaca fascicularis, symptoms, mice, Creutzfeldt-Jakob Disease, cerebellum, cerebral cortex, histopathology, immunohistochemistry, scrapie, strains.

152. Lawler, S. Farmer's research goes against the grain. Trends in Cell Biology 2001 Mar. 11(3): 110. ISSN: 0962-8924
     NAL call no. QH573.T73
     Descriptors: Bovine spongiform encephalopathy, chemically induced, insecticides, organothiophosphate poisoning, cattle, copper and manganese chemistry, prions, intravenous, drug effects.

153. Lledo, Pierre-Marie. Histoire de la vache folle. [‘The history of mad cow.”]. Science, histoire et societe. Paris : Presses Universitaires de France, c2001. xvi, 158, [1] p., ill. In French. ISBN: 2130515738
     NAL call no. SF967.S63 L58 2000
     Descriptors: bovine spongiform encephalopathy, prion diseases, animals prions disease history.

154. Lloyd, S.E.; Onwuazor, O.N.; Beck, J.A.; Mallinson, G.; Farrall, M.; Targonski, P.; Collinge, J.; Fisher, E.M.C. Identification of multiple quantitative trait loci linked to prion disease incubation period in mice Proceedings of the National Academy of Sciences, USA 2001 vol. 98, no. 11, pp. 6279-6283 ISSN: 0027-8424
     NAL call no. 500 P21P
     Descriptors: prion proteins, NvCreutzfeldt-Jakob-disease, scrapie, polymorphism, disease incubation times, bovine spongiform encephalopathy quantitative trait loci, prion protein gene.

155. Lloyd, T.; American Agricultural Economics Association. The impact of food scares on beef and inter-related meat markets. [S.l. : s.n. 2001?]. "Selected paper, American Agricultural Economics Association annual meeting, Chicago, IL, August 5-August 8, 2001."
     NAL call no. HD9421.4.I47 2001
     Descriptors: meat industry and trade, meat inspection, BSE, Great Britain.

156. Lorenzen, S. BSE ist keine Naturkatastrophe, sondern ein menschengemachtes Problem: Wie lasst es sich in Wurde losen. BSE is not a natural disaster, but a man-made problem: how can the BSE crisis be solved in dignity? Fleischwirtschaft. 2001, 81: 4, 127-133; Many ref. ISSN: 0015-363X. In German with an English summary.
     NAL call no. 280.38 F62
     Descriptors: BSE, bovine spongiform encephalopathy, disease control, public health concerns.

157. Lucker, E.; Horlacher, S.; Eigenbrodt, E. Brain in human nutrition and variant Creutzfeldt-Jakob disease risk (vCJD): detection of brain in retail liver sausages using cholesterol and neuron specific enolase (NSE) as markers. British Journal of Nutrition 2001 Aug. 86 Suppl 1: S115-S119. ISSN: 0007-1145
     Abstract: No information is available about the consumption of brain via meat products. With respect to the new variant of Creutzfeldt-Jakob disease (vCJD) and the presumed food-borne transmission of bovine spongiform encephalopathy (BSE) to humans, a preliminary survey for brain and/or spinal cord (tissues of the central nervous system, CNS) was conducted. We applied a previously developed integrated procedure using cholesterol and neuron specific enolase (NSE) as markers. Quantification of cholesterol had to be backed up by NSE immunochemistry in order to account for low specificity and relatively high variances. Out of 126 high-quality finely graded liver sausages, five samples (4 %) showed positive NSE immunoresponses. In four of these samples a transgression of the normal maximum cholesterol content was obtained. The identification of such a considerable number of CNS-positive sausages indicates that brain consumption is not as rare as previously assumed. Overall, the present integrated method could be successfully applied for the detection of CNS in heat-treated meat products. Its routine application in official food control would deter illegal practice and thus help to control transmissible spongiform encephalopathies.
     NAL call no. 389.8 B773
     Descriptors: detection method, brain material, bovine-based meat products, food screening, contamination control, cholesterol and neuron specific enolase.

158. Lundberg, P. O. Annu bara borjan av galna ko-sjukan? Creutzfeldt-Jakobs sjukdom och andra prionsjukdomar: nulaget. [Still a small problem with the mad cow disease? Creutzfeldt-Jakob disease and other prion diseases: current status] Lakartidningen. 2001 Jan 10. 98(1-2): 19-24 ISSN: 0023-7205. In Swedish.
     Abstract: This review is based on recent published research on the BSE/CJD/vCJD problem mainly from UK, Germany and France. The situation in Sweden seems to be fortunate for several reasons. The use of meat and bonemeal as animal fodder was forbidden in this country 13 years ago. Sweden has not had any sheep with scrapie for many years. No animals with BSE have so far been found in our country. The incidence of sporadic CJD in this country followed retrospectively from 1985 to 1996 and prospectively from 1997 to 1999 has been around 1.2 per million per year with no significant increase. Only few cases of familial CJD are known. No patient with iatrogenic CJD has ever been found. The use of growth hormone derived from human pituitary glands was abandoned in 1985 when recombinant human growth hormone became available. So far there is no indication that any of the CJD cases diagnosed in Sweden has been of the vCJD type, the one linked to BSE. However, as the incubation period for prion diseases is very long and the Swedes are frequent travelers there is a risk that people from our country could have contracted vCJD through consuming meat products in countries with BSE. As a precaution the consumption of brain, spinal cord, lymphatic tissue, lungs, and gastrointestinal tract should be avoided. Human pituitary derived growth hormone is still available in some countries and might be illegally imported into Sweden.
     Descriptors: Review article, BSE, NvCreutzfeldt-Jakob Disease, feed ingredients, incidence, disease free, scrapie, battle, sheep, travel risks.

159. Lundberg, P.O. Far, kor--och slakt? [Sheep, cattle--and slaughtering?] Lakartidningen. 2001 Feb 28. 98(9): 990-991 ISSN: 0023-7205. In Swedish.
     Descriptors: sheep, cattle, slaughtering issues, BSE, scrapie.

160. MacKnight, C. Clinical implications of bovine spongiform encephalopathy. Clinical Infectious Diseases. 2001 Jun 15. 32(12): 1726-1731 ISSN: 1058-4838
     Abstract: Bovine spongiform encephalopathy (BSE) is a new prion disease that was first identified in the United Kingdom in 1987. Its appearance was likely caused by changes in the rendering process used to produce a meat and bone supplement for cattle, changes that allowed this prion to enter the bovine food supply. Despite measures that were made to reduce the risk to humans, a new variant of Creutzfeldt-Jakob disease appeared in the mid-1990s and has been linked to BSE. Although the extent of the disease's impact on humans is not yet known, current estimates predict that there will be 136,000 cases of this fatal disease by the year 2040. The risk to humans of medications produced with bovine materials, gelatin, and blood transfusion is unknown.
     NAL call no. RC111 R4
     Descriptors: BSE, risk to human health, NvCreutzfeldt-Jakob-Disease, UK, disease incidence projections, transmission risks of bovine-based pharmaceuticals and other materials, blood transfusions.

161. Magdzik,W. Encefalopatie gabczaste (choroby prionowe) zwierzat. [Spongiform encephalopathy (prion diseases) in animals] Przeglad Epidemiologiczny . 2001. 55(1 Suppl 2): 71-74. ISSN: 0033-2100. In Polish.
     Descriptors: prion diseases, animal incidence, BSE, scrapie.

162. Maissen, M.; Roeckl, C.; Glatzel, M.; Goldmann, W.; Aguzzi, A. Plasminogen binds to disease-associated prion protein of multiple species. Lancet. 2001 Jun 23. 357(9273): 2026-2028 ISSN: 0140-6736
     NAL call no. 448.8 L22
     Descriptors: prion protein, transmissible spongiform encephalopathies, biochemistry.

163. Mangen, M.J.J.; Burrell, A.M. Decomposing preference shifts for meat and fish in the Netherlands. Journal of Agricultural Economics. 2001, 52: 2, 16-28; 30 ref. ISSN: 0021-857X.
     NAL call no. 281.9 AG8
     Descriptors: beef, BSE, bovine spongiform encephalopathy, consumer preferences, elasticities, fish, meat products, pork, poultry meat, consumer preferences, demand changes, Netherlands.

164. Manolakou, K.; Beaton, J.; McConnell, I.; Farquar, C.; Manson, J.; Hastie, N.D.; Bruce, M.; Jackson, I.J. Genetic and environmental factors modify bovine spongiform encephalopathy incubation period in mice. Proc Natl Acad Sci USA. Washington, D.C. : National Academy of Sciences, June 19, 2001. v. 98 (13) p. 7402-7407. ISSN: 0027-8424
     Abstract: The incubation period (IP) and the neuropathology of transmissible spongiform encephalopathies (TSEs) have been extensively used to distinguish prion isolates (or strains) inoculated into panels of inbred mouse strains. Such studies have shown that the bovine spongiform encephalopathy (BSE) agent is indistinguishable from the agent causing variant Creutzfeldt-Jakob disease (vCJD), but differs from isolates of sporadic CJD, reinforcing the idea that the vCJD epidemic in Britain results from consumption of contaminated beef products. We present a mouse model for genetic and environmental factors that modify the incubation period of BSE cross-species transmission. We have used two mouse strains that carry the same prion protein (PrP) allele, but display a 100-day difference in their mean IP following intracerebral inoculation with primary BSE isolate. We report genetic effects on IP that map to four chromosomal regions, and in addition we find significant factors of host environment, namely the age of the host's mother, the age of the host at infection, and an X-cytoplasm interaction in the host.
     NAL call no. 500 N21P
     Descriptors: mice, bovine spongiform encephalopathy. isolation, cattle brain, prepatent period. duration, alleles, animal proteins, application methods, animal models, man, foodborne diseases, genetic effects, disease transmission, age, haplotypes, major histocompatibility complex. quantitative traits. loci.

165. Marouby, H. La consommation de viandes dans l'Union Europeenne: la situation avant la crise. [The consumption of meat in the European Union: the situation before the crisis.] Techni Porc. 2001, 24: 1, 3-4. ISSN: 0181-6764
     NAL call no. SF391.T4
     Descriptors: meat, food consumption, pork, BSE, bovine spongiform encephalopathy, cattle diseases, prion diseases.

166. Masters, C.L. The emerging European epidemic of variant Creutzfeldt-Jakob disease and bovine spongiform encephalopathy: lessons for Australia. Medical Journal of Australia, 2001 Feb 19. 174(4): 160-161 ISSN: 0025-729X
     Descriptors: BSE, NvCreutzfeldt-Jakob Disease, zoonotic diseases, health risks, trade concerns, disease control.

167. Mastrangelo, P.; Westaway, D. The prion gene complex encoding PrP(C) and Doppel: insights from mutational analysis. Gene. 2001 Sep 5. 275(1): 1-18 ISSN: 0378-1119
     Abstract: The prion protein gene, Prnp, encodes PrP(Sc), the major structural component of prions, infectious pathogens causing a number of disorders including scrapie and bovine spongiform encephalopathy (or BSE). Missense mutations in the human Prnp gene cause inherited prion diseases such as familial Creutzfeldt-Jakob disease. In uninfected animals Prnp encodes a glycophosphatidylinositol (GPI)-anchored protein denoted PrP(C) and in prion infections PrP(C) is converted to PrP(Sc) by templated refolding. Though Prnp is conserved in mammalian species, attempts to verify interactions of putative PrP binding proteins by genetic means have proven frustrating and the ZrchI and Npu lines of Prnp gene-ablated mice (Prnp(0/0) mice) lacking PrP(C) remain healthy throughout development. This indicates that PrP(C) serves a function that is not apparent in a laboratory setting or that other molecules have overlapping functions. Current possibilities involve shuttling or sequestration of synaptic Cu(II) via binding to N-terminal octapeptide residues and/or signal transduction involving the fyn kinase. A new point of entry into the issue of prion protein function has emerged from identification of a paralogue, Prnd, with 24% coding sequence identity to Prnp. Prnd lies downstream of Prnp and encodes the doppel (Dpl) protein. Like PrP(C), Dpl is presented on the cell surface via a GPI anchor and has three alpha-helices: however, it lacks the conformationally plastic and octapeptide repeat domains present in its well-known relative. Interestingly, Dpl is overexpressed in the Ngsk and Rcm0 lines of Prnp(0/0) mice via intergenic splicing events. These lines of Prnp(0/0) mice exhibit ataxia and apoptosis of cerebellar cells, indicating that ectopic synthesis of Dpl protein is toxic to central nervous system neurons: this inference has now been confirmed by the construction of transgenic mice expressing Dpl under the direct control of the PrP promoter. Remarkably, Dpl-programmed ataxia is rescued by wild-type Prnp transgenes. The interaction between the Prnp and Prnd genes in mouse cerebellar neurons may have a physical correlate in competition between Dpl and PrP(C) within a common biochemical pathway that when mis-regulated leads to apoptosis.
     NAL call no. QH442.A1G4
     Descriptors: PrP, prion protein functions, missence mutations, mouse models (ZrchI, Npu, Ngsk a Rcm0) transgenic mouse model, doppel proteins.

168. Massara, F.; Scavone, M.; Ferraro, A. Infezioni emergenti e sanita pubblica. [Emergent infections and public health.] Obiettivi e Documenti Veterinari. 2001, 22: 1, 61-64. ISSN: 0392-1913. In Italian.
     Descriptors: foodborne zoonotic diseases, BSE, bovine spongiform encephalopathy, Escherichia coli, disease prevention, public health concerns, salmonellosis, listeriosis, toxoplasmosis, disease control and prrevention, disease-transmission, domestic animals, animal based foods and products.

169. Mazzocchi, M.; Heckelei, T. (ed.); Witzke, H.P. (ed.); Henrichsmeyer, W. Econometric methods for evaluating consumer response to food scares: a structural approach. Agricultural sector modelling and policy information systems. Proceedings of the 65^th European Seminar of the European Association of Agricultural Economists EAAE, Bonn, Germany, 29-31-March, 2000. 2001, 111-120; 36 ref. Wissenschaftsverlag Vauk Kiel KG; Kiel; Germany. ISBN: 3-8175-0329-6
     Descriptors: BSE, bovine spongiform encephalopathy, case studies, food demand models demand functions, food consumption, meat, EM algorithm, Kalman filter.

170. Mazzoni, I.E.; Ledebur, H; Cashman, N. Signal transduction mechanisms linked to prion protein. Society for Neuroscience Abstracts. 2001; 27 (1): 112. ISSN: 0190-5295. 31st Annual Meeting of the Society for Neuroscience, San Diego, California, USA, November 10-15, 2001
     NAL call no. QP351.S6
     Descriptors: cortical postmitotic cultures, bse, Creutzfeldt-Jakob disease, PrP knockout mice, concavalin A, calcium flux, tyrosine phosphorylation, splenocytes.

171. McDonnell, K.; Desmond, J.; Leahy, J.J.; Howard, Hildige R.; Ward, S. Behavior of meat and bonemeal/peat pellets in a bench scale fluidised bed combustor. Energy Oxford. 2001, 26: 1, 81-90; 11 ref. ISSN: 0360-5442
     Descriptors: BSE, animal by-product disposal, fluidised bed combustor, combustion tests, pellets with meat and bone meal, alternative energy source.

172. McGill, I. Phillips report and the origin of BSE. Veterinary Record. 2001 Jan 13. 148(2): 60 ISSN: 0042-4900
     NAL call no. 41.8 V641
     Descriptors: Bovine spongiform encephalopathy, etiology, cattle, mutation, nerve tissue proteins, genetics, prions.

173. Mead, Simon; Mahal, Sukhvir P.; Beck, John; Campbell, Tracy; Farrall, Martin; Fisher, Elizabeth; Collinge, John. Sporadic: But not variant: Creutzfeldt-Jakob disease is associated with polymorphisms upstream of PRNP exon 1. American Journal of Human Genetics. December, 2001; 69 (6): 1225-1235. ISSN: 0002-9297.
     NAL call no. QH431.A1A54
     Descriptors: humans, NvCruetzfeldt-Jakob Disease, etiologies, BSE, prions, PrP gene, codon 129, mouse model, 56 polymorphic sites

174. Menges, T.; Langefeld, T.W.; Krumholz, W.; Hempelmann, G. Ubertragbare spongiforme Enzephalopathien--Anaesthesiologisches und intensivmedizinisches Management. [Transmissible spongiform encephalopathies--anesthetics and intensive care management.] Anasthesiologie, Intensivmedizin, Notfallmedizin, Schmerztherapie-AINS. 2001 Feb. 36(2): 79-89 ISSN: 0939-2661. In German.
     Abstract: The transmissible spongiform encephalopathies (TSE) are known to affect humans and various animals. The bovine spongiform encephalopathy (BSE) and the human Creutzfeldt-Jacob disease (CJD) are among the most notable degenerative disorders caused by prions. Considering the BSE epidemic and the description of a new variant of Creutzfeldt-Jacob disease (nvCJD), which is probably related to bovine spongiform encephalopathy, TSE have recently gained a lot of public attention. Although the causative factors (prions, viruses) are still under discussion, none of the present concepts are explanatory for all aspects of the human CJD. CJD may present as a sporadic, genetic, or infectious illness and there is now considerable concern that bovine prions may have been passed to humans. To exclude transmission of CJD via medical products and instruments, the effectiveness of cleaning, disinfection and sterilization procedures must be firmly established. This manuscript presents an overview to anaesthesiology and intensive care medicine of recommended inactivation procedures and assessed these procedures in the light of the inactivation of prions.
     Descriptors: TSE’s, BSE, CJD, NvCreutzfeldt-Jacob Disease, prions, disinfection/inactivations procedures, anesthetic and analgesic equipment.

175. Miele, G.; Manson, J.; Clinton, M. A novel erythroid-specific marker of transmissible spongiform encephalopathies. Nature Medicine. 2001 Mar. 7(3): 361-364 ISSN: 1078-8956
     Abstract: Transmissible spongiform encephalopathies (TSE) are a group of invariably fatal neurodegenerative diseases and include scrapie in sheep, bovine spongiform encephalopathy (BSE) in cattle, chronic wasting disease in deer and elk, and Kuru disease, Creutzfeldt-Jakob disease (CJD) and variant CJD in humans. The pathological effects of disease occur predominantly in the CNS (central nervous system), where common hallmarks include vacuolation, gliosis, accumulation of a protease-resistant, abnormally folded isoform of the prion protein (PrPSc) and neuronal cell death. Lack of understanding of the molecular mechanisms underlying disease pathogenesis, particularly in non-CNS tissues, means that there are currently no effective strategies for early diagnosis or therapeutic intervention of TSEs. Here we report the first identification of a molecular marker that is easily detectable in readily accessible tissues. We demonstrate that a dramatic decrease in expression of a transcript specific to erythroid lineage cells is a common feature of TSEs. Our findings indicate a previously unrecognized role for involvement of the erythroid lineage in the etiology of TSE pathogenesis and should provide a new focus for research into diagnostic and therapeutic strategies.
     Descriptors: transmissible spongiform encephalopathies, molecular marker, pathogenesis, research and diagnostic directions, non-CNS tissues, early disgnosis, prion diseases.

176. Miles, S.; Frewer, L.J. Investigating specific concerns about different food hazards. Food Quality and Preference. 2001, 12: 1, 47-61; 35 ref. ISSN: 0950-3293
     NAL call no. TX367.F66
     Descriptors: food safety, food hazards, consumer attitudes, public health concerns, microbial and pesticide contamination of food, genetic engineered; foods, BSE, bovine spongiform encephalopathy, risk factors, laddering techniques.

177. Murphy, C.; Breen, C.; Rogers, M.; Giese, M. Interferon gamma and prostaglandin in BSE-infected cattle. Cytokine. 2001 Feb 7. 13(3): 169-173 ISSN: 1043-4666
     Abstract: Suspect field cases of BSE infection (displaying clinical signs) were examined for possible alterations of cytokine/autacoid plasma levels and were compared to control cases (not displaying clinical signs of BSE infection). Interferon gamma (IFN-gamma) plasma levels were demonstrated as being elevated in all suspected field cases of BSE infection (irrespective of BSE status; determined via postmortem histopathological examination). We demonstrated that plasma IFN-gamma levels were significantly (P<0.005) higher in suspect cases of BSE infection than in control cases. BSE-positive prostaglandin-E(2), (PGE(2)) plasma levels were demonstrated as being elevated 1.25-fold above BSE-negative cases and 2.22-fold above control cases. No significant (P>0.5) increase in PGE(2)plasma levels was recorded between BSE-positive and -negative. IFN-gamma and PGE(2)plasma levels were examined using commercially available ELISA assay. The results presented in this publication are the first demonstration of alteration in immune state in animals with BSE.
     NAL call no. QR185.C95C986
     Descriptors: bovine spongiform encephalopathy, cattle, IFN-gamma levels, field cases, altered immune status.

178. Murray, G. Feed controls -- stopping BSE (Mad Cow Disease). Agnote NSW Agriculture. 2001, No. DAI-227(1st Ed.), 2 pp. NSW Agriculture; Orange; Australia. ISSN: 1034-6848
     NAL call no. SF55 A8 A36
     Descriptors: BSE, bovine spongiform encephalopathy, cows, feeds, labeling controls, meat and bone meal, prion diseases, control measures.

179. Narang, H.K. A critical review of atypical cerebellum-type Creutzfeldt-Jakob disease: its relationship to "new variant" CJD and bovine spongiform encephalopathy. Experimental Biology and Medicine (Maywood). 2001 Jul. 226(7): 629-639 ISSN: 1535-3702
     Abstract: Shortly after the appearance of bovine spongiform encephalopathy (BSE), Creutzfeldt-Jakob disease (CJD) was identified in young patients with nonclassical presentation such as difficulty in balancing and ataxia. The classical CJD in older patients starts with dementia. To distinguish between the two types, CJD in young persons has been termed "new variant" (nvCJD). The distinguishing features of classical CJD include initial presentation with dementia, confluent spongiform changes are very unusual in the cerebellum, and PrP plaques are rarely observed. For nvCJD, initially, difficulty with balancing and ataxia occurs, confluent spongiform changes are seen in the cerebellum, and a large number of PrP plaques are seen. The Icelandic observation of sheep scrapie revealed a predominantly ataxic form of scrapie, termed Type II, rather than the itchy form termed Type I. Both types have been known to exist in Europe. Since the clinical signs of Type II scrapie in sheep with trembling and ataxia are similar to those seen in BSE and nvCJD, this suggests that Type II is the cause of BSE and nvCJD. Over 8 years, from 1989 to 1996, I examined the clinical histories of 33 CJD cases aged between the ages of 18 and 84. Six under the age of 40 and 15 over the age of 40 had leading clinical features such as difficulty in balancing and ataxia similar to those seen in the young cases classified as "nvCJD." Brains were examined from the six of 15 cases over the age of 40, which revealed similar pathology to that seen in young patients classified as "nvCJD." These findings suggest that all age groups are susceptible to the strain of the agent derived from BSE cattle.
     NAL call no. QH301 E9
     Descriptors: BSE, NvCreutzfeldt-Jakob Disease, young humans, disease comparison, brain tissues, cerebellum.

180. Narang, H.K. Lingering doubts about spongiform encephalopathy and Creutzfeldt-Jakob disease. Experimental Biology and Medicine (Maywood). 2001 Jul. 226(7): 640-652 ISSN: 1535-3702
     Abstract: Bovine spongiform encephalopathy (BSE) is an infectious disease and has been transmitted orally to many other animals, including humans. There is clear evidence of maternal transmission, although disagreement on the source of the BSE agent remains. The current theories link the origin of BSE to common scrapie in sheep. Twenty different strains of the scrapie agent have been isolated from sheep. A search of the literature indicates two distinct clinical syndromes in sheep, both of which have been called scrapie. I have designated these Type I (the common type), which exhibits itchiness and lose their wool, and Type II, which exhibits trembling and ataxia. Sheep inoculated with BSE develop Type II scrapie and they exhibit trembling. When cattle or mink are injected with the Type I strain, only a few will develop a clinical disease. By contrast, no clinical disease has so far been shown in cattle or mink by feeding them with Type I-infected sheep brains. However, either by injecting or feeding with the BSE strain, 100% of calves and mink develop the clinical disease. Evidence suggests that Type II is the cause of BSE. Identical clinical signs of Type II trembling are found in kuru and many of the recent cases of Creutzfeldt-Jakob disease. The BSE agent has caused spongiform encephalopathies (SEs) in domestic cats, tigers, and in some species of ruminants in zoos. The nature of the BSE agent remains unchanged when passaged through a range of species, irrespective of their genetic make up, demonstrating that variations in the host PrP gene are not a major factor in the susceptibility to the BSE agent. Since more than 85 zoo animals of many species have been diagnosed with SEs, from these studies it seems reasonable to conclude that the BSE agent can infect almost all mammalian species, including humans. For eradication of BSE and to reduce the risk of infection to humans, the development of a vaccine against BSE is suggested. Such a possibility should be fully explored.
     NAL call no. QH301 E9
     Descriptors: BSE, scrapie agents, Type I and Type II clinical syndromes, sheep, mink, cattle, Type comparisons, etiology, zoo animals, mammalian susceptibility.

181. Negro, A.; Ballarin, C.; Bertoli, A.; Massimino, M.L.; Sorgato, M.C. The metabolism and imaging in live cells of the bovine prion protein in its native form or carrying single amino acid substitutions. Molecular and Cellular Neurosciences 2001 Mar. 17(3): 521-538. ISSN: 1044-7431
     Abstract: Prion diseases are probably caused by an abnormal form of a cellular glycoprotein, the prion protein. Recent evidence suggests that the prion strain causing BSE has been transmitted to humans, thereby provoking a variant form of Creutzfeldt-Jacob disease. In this work, we analyzed the behavior of normal and malformed isoforms of the bovine PrP in transfected mammalian cell lines. Biochemical and immunocytochemical assays were complimented with imaging of live cells expressing fusion constructs between PrP and GFP. Bovine homologues of human E200K and D178N (129M) mutations were used as models of pathogenic isoforms. We show that the GFP does not impair the metabolism of native and mutant bPrPs and is thus a valid marker of PrP cellular distribution. We also show that each amino acid replacement provokes alterations in the cell sorting and processing of bPrP. These are different from those ascribed to both murine mutant homologues. However, human and bovine PrPs carrying the D178N genotype had similar cellular behavior.
     Descriptors: prion protein, behavior of normal and isoforms, bovine PrP, transfected cell lines, GFP, E200K, D178N (129M).

182. O'-Farrell, K.; Dillon, P.; Mee, J.; Crosse, S.; Nolan, M.; Byrne, N.; Reidy, M.; Flynn, F.; Condon, T. Strategy for restocking of Moorepark after depopulation following bovine spongiform encephalopathy. Irish Veterinary Journal. 2001, 54: 2, 70-75; 5 ref. ISSN: 0368-0762.
     NAL call no. 41.8 IR4
     Descriptors: BSE, bovine spongiform encephalopathy, cattle diseases, animal-health, dairy cattle, disease control, Moorepark Centre Farm restocking strategy, herd health status monitoring.

183. Office International des Epizooties. Foot and mouth disease in Turkey, Peninsular Malaysia and Kazakhstan; Classical swine fever in Spain and Slovakia; Bovine spongiform encephalopathy in Greece. Disease Information Office International des Epizooties. 2001, 14: 27, 165-172. ISSN: 1012-5329
     NAL call no. SF781 D57
     Descriptors: BSE, bovine spongiform encephalopathy, disease incidence statistics, epidemiology, foot and mouth disease, outbreaks, swine fever

184. O'Mara F.; O' Doherty J. The effect of removing fishmeal and meat-and-bone meal from animal diets. Irish Veterinary Journal. 2001, 54: 5, 244-245. ISSN: 0368-0762
     NAL call no. 41.8 IR4
     Descriptors: BSE, animal protein in feeds, alternative replacements, costs, nutritional values, Ireland.

185. Oomkes, C.; van Knapen, F. Cows, cats, and FSE: death penalty justified? Veterinary Quarterly 2001 Jan. 23(1): 51-52 ISSN: 0165-2176
     Abstract: Transmissible spongiform encephalopathies affect a number of mammalian species. The most common spongiform encephalopathies are scrapie in sheep and Bovine Spongiform Encephalopathy (BSE) in cattle. Feline Spongiform Encephalopathy (FSE) is a related disorder in domestic cats. Because of the link between BSE and FSE, cats are put on a par with cattle, in terms of politics and regulations. In the Netherlands, when a case of BSE is found on a farm, not only the ruminants, but also the cats are taken away for post-mortem examination. So far, the cats examined have always been negative for FSE. There are no scientific reasons for destroying the cats on farms where BSE has been found.
     NAL call no. SF601 V46
     Descriptors: BSE, feline spongiform encephalopathy, euthanizing cats, farms positive for BSE, incidence of disease.

186. Operativi gli interventi per fronteggiare la Bse. [Financial interventions to cope with BSE.] Informatore Agrario. 2001, 57: 12, 13. ISSN: 0020-0689. In Italian.
     NAL call no. 281.8 IN32
     Descriptors: bovine spongiform encephalopathy, cattle diseases, nervous system diseases, prevention, prion diseases, support measures.

187. Overgaauw, P. A. BSE en petfood: is diervoeding wel veilig? [BSE and pet food: is animal feed safe?] Tijdschrift voor Diergeneeskunde. 2001 Apr 15. 126(8): 292 ISSN: 0040-7453. In Dutch.
     NAL call no. 41.8 T431
     Descriptors: BSE contaminated pet foods, cats, transmissible spongiform encephalopathies.

188. Pallaroni, Lea; Bjorklund, Erland; von-Holst, Christoph; Unglaub, Wolfgang. Determination of rendering plant sterilization conditions using a commercially available ELISA test kit developed for detection of cooked beef. Journal of AOAC-International. November-December, 2001; 84 (6): 1884-1890. ISSN: 1060-3271.
     NAL call no. S583 A7
     Descriptors: BSE, enzyme-linked immunosorbent assay (ELISA) test kit, cooked beef/pork detection, rendering process, laboratory conditions, temperature, time, particle size and meat composition.

189. Paukstadt, W. BSE-Rinder in Deutschland. Mussen wir bald auch mit vCJK-Patienten rechnen? [BSE-cattle in Germany. Must we soon also count on vCJK patients?] MMW-Fortschritte-der-Medizin. 2001 Jan 25. 143(4): 12-13 ISSN: 1438-3276. In German.
     Descriptors: Creutfeldt-Jakob Syndrome transmission, brain pathology, cattle, diagnosis, Germany, risk factors.

190. Peretz, D.; Williamson, R.A.; Kaneko, K.; Vergara, J.; Leclerc, E.; Schmitt-Ulms, G.; Mehlhorn, I. R.; Legname, G.; Wormald, M. R.; Rudd, P.M.; Dwek, R.A.; Burton, D.R.; Prusiner, S.B. Antibodies inhibit prion propagation and clear cell cultures of prion infectivity. Nature. 2001 Aug 16. 412(6848): 739-743 ISSN: 0028-0836
     Abstract: Prions are the transmissible pathogenic agents responsible for diseases such as scrapie and bovine spongiform encephalopathy. In the favoured model of prion replication, direct interaction between the pathogenic prion protein (PrPSc) template and endogenous cellular prion protein (PrPC) is proposed to drive the formation of nascent infectious prions. Reagents specifically binding either prion-protein conformer may interrupt prion production by inhibiting this interaction. We examined the ability of several recombinant antibody antigen-binding fragments (Fabs) to inhibit prion propagation in cultured mouse neuroblastoma cells (ScN2a) infected with PrPSc. Here we show that antibodies binding cell-surface PrPC inhibit PrPSc formation in a dose-dependent manner. In cells treated with the most potent antibody, Fab D18, prion replication is abolished and pre-existing PrPSc is rapidly cleared, suggesting that this antibody may cure established infection. The potent activity of Fab D18 is associated with its ability to better recognize the total population of PrPC molecules on the cell surface, and with the location of its epitope on PrPC. Our observations support the use of antibodies in the prevention and treatment of prion diseases and identify a region of PrPC for drug targeting.
     NAL call no. 472 N21
     Descriptors: prions, antibody antigen-binding fragments, Fab D18, cultured mouse neuroblastoma cells, prevention and treatment strategy.

191. Pettitt, R. G. Traceability in the food animal industry and supermarket chains. Revue Scientifique et Technique International Office of Epizootics. 2001 Aug. 20(2): 584-597 ISSN: 0253-1933
     Abstract: Since the 1950s, consumers in the United Kingdom (UK) have learned to expect cheap, but safe food. A number of incidents in the 1980s and 1990s caused public alarm and loss of confidence in the role of producers and the Government in the food supply. This review examines the impact of recent food scares in the UK, where scrutiny of the food industry has led to the introduction of new controls at all stages of production. Animal feed manufacture, livestock production, slaughter and the use or disposal of animal by-products are now controlled in ways unimagined prior to the identification of bovine spongiform encephalopathy (BSE) in the late 1980s. Traceability has become an important issue for consumers and, by proxy, for the multiple retailers that service consumer needs. Retailers have increasingly managed the food chain to ensure high standards that can be proven by audit. The retailers have also found that a commercial advantage can be gained from certain aspects of source verification. In order to maximise sales in a depressed market, producer groups have themselves developed a multiplicity of assurance schemes.
     NAL call no. SF781.R4
     Descriptors: food scares, animal product source verification, assurance schemes, UK, review, impact of BSE on animal production and marketing.

192. Perez-Bonilla, Q. Bovine spongiform encephalopathy in Spain; West Nile fever in France; Foot and mouth disease in Swaziland and South Africa; Newcastle disease in Comoros. Disease Information Office International des Epizooties. 2001, 14: 2, 6 pp. ISSN: 1012-5329
     NAL call no. SF781 D57
     Descriptors: BSE, bovine spongiform encephalopathy, foot and mouth disease, livestock diseases, Newcastle disease, West Nile fever, disease survey and incidence. France, South Africa, Comoros.

193. Pistoi, S. Italians drop beef as first cow tests positive. Nature. 2001 Jan 25. 409(6819): 441 ISSN: 0028-0836
     NAL call no. 472 N21
     Descriptors: Cattle, bovine spongiform encephalopathy, diagnosis, Italy, meat.

194. Podger, G. BSE report. Lancet. 2001 Feb 3. 357(9253): 397-398 ISSN: 0140-6736
     NAL call no. 448.8 L22
     Descriptors: bovine spongiform encephalopathy, epidemiology, incidence.

195. Pohlenz J.; Kamphues J. (ed.); Flachowsky, G. Zur Pathogenese der transmissiblen spongiformen Enzephalopathien (TSE/BSE und Scrapie). [Pathogenesis of transmissible spongiform encephalopathies (TSE/BSE and scrapie).] Animal nutrition - resources and future developments. Workshop on Sustainable Animal Production, 15-16 June 2000, EXPO 2000, Hannover, Germany. Landbauforschung Volkenrode, Sonderheft. 2001, No. 223, 142-151; 1 ref. ISBN: 0376-0723. In German with an English summary.
     Descriptors: BSE, bovine spongiform encephalopathy, NvCreutzfeldt Jakob disease, prion disease course, infection process, meat and bone meal products of rendering, scrapie, TSE, prion resistance to degradation, Germany.

196. Polak, M.P.; Zmudzinski, J.F. BSE w Europie -- dane epizootyczne. [BSE in Europe -- epizootiological data.] Medycyna Weterynaryjna. 2001, 57: 4, 228-232; 12 ref. ISSN: 0025-8628. In Polish with an English summary.
     NAL call no. 41.8 M463
     Descriptors: BSE, bovine spongiform encephalopathy, meat and bone meal, epizoological data.

197. Polak, M.P.; Zmudzinski, J.F. BSE jako zoonoza. [BSE as zoonosis.] Medycyna Weterynaryjna. 2001, 57: 1, 5-8; 30 ref. ISSN: 0025-8628. In Polish with an English summary.
     NAL call no. 41.8 M463
     Descriptors: BSE, bovine spongiform encephalopathy, NvCreutzfeldt Jakob disease; cattle, humans, zoonotic diseases.

198. Prange, H. Stellungnahme Zur Risikoeinschatzung der BSE und zur Bewertung der BSE-Gesellschaftskrise. [Crisis committee for BSE risk evaluation.] Tierarztliche Umschau. 2001, 56: 4, 171-174. ISSN: 0049-3864. In German.
     NAL call no. 41.8 T445
     Descriptors: BSE, bovine spongiform encephalopathy, public health risk factors, Germany.

199. Prusiner, Stanley B. Mad cows, demented people, and the biology of neurodegeneration. Journal of Human Virology. May-June, 2001; 4 (3): 132. 2001 International Meeting of the Institute of Human Virology, Baltimore, Maryland, USA, September 9-13, 2001. ISSN: 1090-9508
     Descriptors: BSE, cattle, spongiform encephalopathies, prions, biochemistry, neurodegenerative diseases, causes.

200. Purdey, M. Does an ultra violet photooxidation of the manganese-loaded/copper-depleted prion protein in the retina initiate the pathogenesis of TSE? Medical Hypotheses 2001 Jul. 57(1): 29-45 ISSN: 0306-9877
     Abstract: Ecosystems supporting clusters of sporadic transmissible spongiform encephalopathy (TSE) are characterized by common properties of high-manganese/low-copper, zinc, selenium mineral status, and high-altitude/snow-covered/pre-Cambrian mountain terrain where above-average intensities of ultra violet/ozone oxidants are prevalent. Cell culture trials have confirmed the hypothesis that manganese (Mn) substitutes at Prion Protein's (PrP's) vacated copper (Cu) domain, whereupon PrP loses its Cu-mediated antioxidant function, transforming into a protease-resistant misfolded isoform that aggregates into fibril 'tombstone' structures - the key hallmark distinguishing TSE central nervous system (CNS) pathology. The cellular localisation of PrP suggests PrP serves a 'front line' contributory role in neutralizing radicals generated by incoming environmental oxidants, whilst an intensive expression of PrP messenger ribonucleic acid (mRNA) in the retina, melanocytes, epidermis, etc., suggests PrP performs a key antioxidant role as a 'photooxidative shock absorber'; binding of porphyrin IX, Congo red and other photosensitisers to PrPc suggests PrPc serves as an integral associate of the porphyrin/melanin chromophore electron transfer chain; thereby serving as a quencher of singlet O2/superoxide generated by photoenergised chromophores/xeno photosensitisers. It is proposed that sporadic TSE pathogenesis is initiated in the retina of environmentally/genetically predisposed individuals via a two-stage chronic toxic process - Mn substitution at PrP's Cu domain forming a stable Mn2+-PrP complex, followed by an ultra violet in situ photo-oxidization of the Mn2+ component; whereby the latent 'Jekyll and Hyde' capacity of the Mn2+-PrP conjugate is activated into the fully fledged, 'infectious' lethal auto-oxidizing, Mn3+-PrP 'prion' agent. Thus, PrPc's Cu-mediated antioxidant function is replaced by a Mn3+-mediated autooxidant dysfunction. Could the UK's increased loading of a cocktail of environmental oxidants that penetrated the CNS of the UK bovine (ultra violet microwaves/ozone/systemic cu-chelating insecticides) account for a more virulent Mn4+ mediated acceleration of the TSE degenerative process in Mn-contaminated/genetically predisposed individuals, manifesting as the widespread emergence of new-variant bovine spongiform encephalopathy (BSE)/variant Creutzfeldt-Jacob disease (VCJD)/FSE in younger mammals?
     Descriptors: prions, pathogenesis, environmental factors, copper-mediated antioxidant functions, prion protein isoforms, genetic disposition, BSE, NvCreutzfeldt-Jakob disease, causes.

201. Rabenau, H.F.; Cinatl, J.; Doerr, H.W.; Rabenau, H.F. (ed.); Cinatl, J. (ed.); Doerr, H.W. Prions: a challenge for science,-medicine-and-public-health-system. 2001, viii + 164 pp. Published by: S. Karger AG; Basel; Switzerland. ISBN: 3-8055-7124-0. Includes 11 papers on the current knowledge of prion diseases in man and animals.
     NAL call no. QR1 C66 v. 7
     Descriptors: BSE, bovine spongiform encephalopathy, Creutzfeldt Jakob disease, prion diseases, prion proteins, scrapie, disease theories, strain variations, species barriers, decontamination, epidemiology, human health risks.

202. Race, R.; Raines, A.; Raymond, G. J.; Caughey, B.; Chesebro, B. Long-term subclinical carrier state precedes scrapie replication and adaptation in a resistant species: analogies to bovine spongiform encephalopathy and variant Creutzfeldt-Jakob disease in humans. Journal of Virology. 2001 Nov. 75(21): 10106-10112 ISSN: 0022-538X
     Abstract: Cattle infected with bovine spongiform encephalopathy (BSE) appear to be a reservoir for transmission of variant Creutzfeldt-Jakob disease (vCJD) to humans. Although just over 100 people have developed clinical vCJD, millions have probably been exposed to the infectivity by consumption of BSE-infected beef. It is currently not known whether some of these individuals will develop disease themselves or act as asymptomatic carriers of infectivity which might infect others in the future. We have studied agent persistence and adaptation after cross-species infection using a model of mice inoculated with hamster scrapie strain 263K. Although mice inoculated with hamster scrapie do not develop clinical disease after inoculation with 10 million hamster infectious doses, hamster scrapie infectivity persists in brain and spleen for the life span of the mice. In the present study, we were surprised to find a 1-year period postinfection with hamster scrapie where there was no evidence for replication of infectivity in mouse brain. In contrast, this period of inactive persistence was followed by a period of active replication of infectivity as well as adaptation of new strains of agent capable of causing disease in mice. In most mice, neither the early persistent phase nor the later replicative phase could be detected by immunoblot assay for protease-resistant prion protein (PrP). If similar asymptomatic carriers of infection arise after exposure of humans or animals to BSE, this could markedly increase the danger of additional spread of BSE or vCJD infection by contaminated blood, surgical instruments, or meat. If such subclinical carriers were negative for protease-resistant PrP, similar to our mice, then the recently proposed screening of brain, tonsils, or other tissues of animals and humans by present methods such as immunoblotting or immunohistochemistry might be too insensitive to identify these individuals.
     NAL call no. QR360.A1J6
     Descriptors: mouse animal models, scrapie, prion protein, immunoblotting, brain, spleen, hamster scrapie strain 263K, immunoblot assay, subclinical infection.

203. Raiden, R.M.; Sumner, S.S.; Pierson, M.D. Bovine spongiform encephalopathy: a brief overview. Dairy Food Environ Sanit. Des Moines, IA : International Association of Milk, Food and Environmental Sanitarians, Inc. Aug 2001. v. 21 (8) p. 685-690. ISSN: 1043-3546
     NAL call no. SF221.D342
     Descriptors: bovine spongiform encephalopathy, prion proteins, disease transmission, infections, food contamination, regulations, disease control, etiology, history, symptoms, clinical aspects, feeds, WHO, literature-reviews, UK, USA.

204. Ratzan, S.C. Human risk from eating beef: risk communication gone mad. Proc Agric Outlook Forum. Washington, D.C. : U.S. Dept. of Agriculture, World Agricultural Outlook Board, 2001. p. n/a. Meeting held February 22-23, 2001, Arlington, Virginia. Includes references.
     NAL call no. aHD1755.A376
     Descriptors: food safety, bovine spongiform encephalopathy, risk factors, human health, beef products, communication to the public.

205. Ratzan, S.C. Mad Cow Crisis II: new casualties. Journal of Health Communications. 2001 Jan-Mar. 6(1): 1-2 ISSN: 1081-0730
     Descriptors: Bovine spongiform encephalopathy, transmission, epidemiology, health policy, cattle, England, public health.

206. Revenu agricole 2000: des resultats paradoxaux. [Agricultural income 2000: paradoxal results.] Chambres d'Agriculture. 2001, No. 895, 29-31. ISSN: 0396-7883. In French.
     NAL call no. 14 T69
     Descriptors: France, BSE, effects of Agenda 2000, agricultural-production data, bovine spongiform encephalopathy; cattle diseases, prion diseases

207. Riemann, H. Bovine spongiform encephalopathy and variant Creutzfeldt-Jakob disease. Emerging Infectious Diseases 2001. 7(3 Suppl): 605 ISSN: 1080-6040
     NAL call no. RA648.5 E46
     Descriptors: BSE, issues and concerns, human health risks.

208. Riesner, D.; Rabenau, H.F. (ed.); Cinatl, J. (ed.); Doerr, H.W. The prion theory: background and basic information. Prions: a challenge for science, medicine and public health-system. 2001, 7-20; 48 ref. Published by: S. Karger AG; Basel; Switzerland. ISBN: 3-8055-7124-0
     NAL call no. QR1 C66 v. 7
     Descriptors: BSE, bovine spongiform encephalopathy, Creutzfeldt Jakob Disease; prion diseases, scrapie, disease theories, pathogenesis, description of the disease process.

209. Roos, R.P. Controlling new prion diseases. New England Journal of Medicine, 2001 May 17. 344(20): 1548-1551. ISSN: 0028-4793
     NAL call no. 448.8 N442
     Descriptors: review of US Federal programs, BSE, NvCreutzfeldt-Jakob Disease, scrapie, importation bans, bovine-derived product risks, trade, USDA screening program, killing methods, control of CNS material in meat, ban on ruminant products in feeds.

210. Rossides, S. Meat and bone meal still used in animal feed. Nature. 2001 Nov 8. 414(6860): 147 ISSN: 0028-0836
     NAL call no. 472 N21
     Descriptors: Animal feed, bovine spongiform encephalopathy, etiology, cattle, meat, bone, Japan, Great Britain.

211. Ryder, S. J.; Wells, G. A.; Bradshaw, J.M.; Pearson, G. R. Inconsistent detection of PrP in extraneural tissues of cats with feline spongiform encephalopathy. Veterinary Record. 2001 Apr 7. 148(14): 437-441. ISSN: 0042-4900
     Abstract: Feline spongiform encephalopathy (FSE), a transmissible spongiform encephalopathy or prion disease of cats, first reported in Great Britain in 1990, is believed to result from the consumption of food contaminated by the agent of bovine spongiform encephalopathy (BSE). The accumulation of PrP in non-neural tissues of cats diagnosed as suffering from FSE was investigated by immunohistochemistry. In the majority of the cats no disease-specific PrP was detected in lymphoid tissues. Small amounts of PrP were detected in the spleen of only two of 13 samples examined, in Peyer's patches of one of the two cases for which suitable material was available, but in the myenteric plexus of all four cats in which sections of intestine were examined. In addition PrP immunostaining was found in the kidney of all the cats with FSE whose kidneys were examined.
     NAL call no. 41.8 V641
     Descriptors: FSE, feline spongiform encephalopathy, cats, BSE contaminated food, immunohistochemistry diagnosis, spleen, Peyer’s patches, myenteric plexus, kidney, PrP.

212. Saldums, L. Rapid detection of BSE Food Australia 2001 vol. 54, no. 4, p. 116 ISSN: 1032-5298
     NAL call no. TP368 F662
     Descriptors: scrapie, sheep, BSE, cattle, NvCreutzfeld-Jakob disease, UK, PrP detection.

213. Sarradin, P; Lantier, F. Les moutons sont-ils devenus "fous". [Does mad cow disease occur in sheep?] Biofutur. 2001, No. Hors Serie, 42-46; 21 ref. ISSN: 0294-3506. In French.
     NAL call no. TP248.13 B565
     Descriptors: BSE, French Food Safety Agency recommendations, bovine spongiform encephalopathy, food safety, bans on consumption of sheep intestines, spleen, brain, public health, France.

214. Schiermeier, Q. Testing times for BSE. Nature. 2001 Feb 8. 409(6821): 658-659 ISSN: 0028-0836
     NAL call no. 472 N21
     Descriptors: Bovine spongiform encephalopathy, diagnosis, animal feed, brain chemistry, cattle, disease outbreaks, food contamination, immunologic techniques, meat, mice, prions analysis.

215. Schluter, H.; Doherr, M.; Kramer, M.; Teuffert, J. Notwendigkeit von bestandsbezogenen BSE-Bekampfungsmassnahmen zur Tierseuchenprophylaxe oder zum Verbraucherschutz?! [Is there a need to cull whole BSE-affected herds to prevent disease and protect consumers?] Tierarztliche Umschau. 2001, 56: 7, 344-351; 6 ref. ISSN: 0049-3864. In German with an English summary.
     NAL call no. 41.8 T445
     Descriptors: BSE, bovine spongiform encephalopathy, cattle culling, disease control and prevention, public health concerns.

216. Schreuder, BEC; Geertsma, RE; Keulen, LJM van; Enthoven, P; Oberthur, RC; Koeijer, AA de; Osterhaus, ADME; van Keulen, LJM; de Koeijer, AA; Kamphues, J (ed.); Flachowsky, G. Inactivation of prions by rendering processes. Animal nutrition, resources and future developments. Workshop on Sustainable Animal Production, 15-16 June 2000, EXPO 2000, Hannover, Germany. Landbauforschung Volkenrode, Sonderheft. 2001, No. 223, 130-141; 23 ref. ISSN: 0376-0723; ISBN: 3-933140-47-1. In English with German summaries.
     Descriptors: BSE, bovine spongiform encephalopathy, brain, infectivity, rendering, scrapie.

217. Schudel, A.A.; Gelderen C. van; van Gelderen C. La encefalopatia espongiforme bovina: un enfoque regional. [Bovine spongiform encephalopathy: A regional problem.] Veterinaria Argentina. 2001, 18: 174, 282-289; 3 ref. ISSN: 0326-4629. In Spanish.
     NAL call no. SF604 V463
     Descriptors: BSE, bovine spongiform encephalopathy, cattle diseases, diffusion of information, monitoring, prion diseases, risk assessment, Argentina, South America at risk.

218. Science in a political context. Vet Rec. London : The British Veterinary Association. July 7, 2001. v. 149 (1) p. 1. ISSN: 0042-4900
     NAL call no. 41.8 V641
     Descriptors: foot and mouth disease, bovine spongiform encephalopathy, outbreaks, reports, policy, decision making, UK.

219. Shaked, G.M.; Shaked, Y.; Kariv Inbal, Z.; Halimi, M.; Avraham, I.; Gabizon, R. A protease-resistant prion protein isoform is present in urine of animals and humans affected with prion diseases. J Biol Chem. Bethesda, Md. : American Society for Biochemistry and Molecular Biology. Aug 24, 2001. v. 276 (34) p. 31479-31482. ISSN: 0021-9258
     NAL call no. 381 J824
     Descriptors: cattle, hamsters, man, scrapie, bovine spongiform encephalopathy, Creutzfeldt-Jakob Disease, prion proteins, resistance, proteinases, urine, prions, diagnosis.

220. Shaked, G.M.; Meiner, Z.; Avraham, I.; Taraboulos, A.; Gabizon, R. Reconstitution of prion infectivity from solubilized protease-resistant PrP and nonprotein components of prion rods. Journal of Biological Chemistry. 2001 Apr 27. 276(17): 14324-14328 ISSN: 0021-9258
     Abstract: The scrapie isoform of the prion protein, PrP(Sc), is the only identified component of the infectious prion, an agent causing neurodegenerative diseases such as Creutzfeldt-Jakob disease and bovine spongiform encephalopathy. Following proteolysis, PrP(Sc) is trimmed to a fragment designated PrP 27-30. Both PrP(Sc) and PrP 27-30 molecules tend to aggregate and precipitate as amyloid rods when membranes from prion-infected brain are extracted with detergents. Although prion rods were also shown to contain lipids and sugar polymers, no physiological role has yet been attributed to these molecules. In this work, we show that prion infectivity can be reconstituted by combining Me(2)SO-solubilized PrP 27-30, which at best contained low prion infectivity, with nonprotein components of prion rods (heavy fraction after deproteination, originating from a scrapie-infected hamster brain), which did not present any infectivity. Whereas heparanase digestion of the heavy fraction after deproteination (originating from a scrapie-infected hamster brain), before its combination with solubilized PrP 27-30, considerably reduced the reconstitution of infectivity, preliminary results suggest that infectivity can be greatly increased by combining nonaggregated protease-resistant PrP with heparan sulfate, a known component of amyloid plaques in the brain. We submit that whereas PrP 27-30 is probably the obligatory template for the conversion of PrP(C) to PrP(Sc), sulfated sugar polymers may play an important role in the pathogenesis of prion diseases.
     NAL call no. 381 J824
     Descriptors: PrP 27-30, sulfanated sugar polymers, prion diseases, BSE, CJD, prion infectivity.

221. Shears, P.; Zollers, F.; Hurd, S. Food for thought: what mad cows have wrought with respect to food safety regulation in the EU and UK. British Food Journal. 2001, 103: 1, 63-87; 13 ref. ISSN: 0007-070X
     NAL call no. 389.8 B77
     Descriptors: BSE, bovine spongiform encephalopathy, consumer behavior, Creutzfeldt Jakob disease, epidemiology, EU, UK, food legislation, food policy, food safety regulations.

222. Sigurdson, C.J.; Spraker, T.R.; Miller, M.W.; Oesch, B.; Hoover, E.A. PrP(CWD) in the myenteric plexus, vagosympathetic trunk and endocrine glands of deer with chronic wasting disease. Journal of General Virology. 2001 Oct. 82(Pt 10): 2327-2334 ISSN: 0022-1317
     Abstract: Accumulated evidence in experimental and natural prion disease systems supports a neural route of infectious prion spread from peripheral sites of entry to the central nervous system. However, little is known about prion trafficking routes in cervids with a naturally occurring prion disease known as chronic wasting disease (CWD). In the brain, the pathogenic isoform of the prion protein (PrP(CWD)) accumulates initially in the dorsal motor nucleus of the vagus nerve. To assess whether alimentary-associated neural pathways may play a role in prion trafficking, neural and endocrine tissues from mule deer naturally infected with CWD (n=6) were examined by immunohistochemistry. PrP(CWD) was detected in the myenteric plexus, vagosympathetic trunk, nodose ganglion, pituitary, adrenal medulla and pancreatic islets. No to scant PrP(CWD) staining was detected in other nerves or ganglia (brachial plexus, sciatic nerve, gasserian ganglion, coeliac ganglion, cranial cervical ganglion, spinal nerve roots) of CWD-positive deer and no PrP(CWD) was detected in nerves or endocrine tissues from 11 control deer. These findings suggest that: (i) transit of PrP(CWD) in nerves, either centrifugally or centripetally, is one route of prion trafficking and organ invasion and (ii) endocrine organs may also be targets for cervid pathogenic prion accumulation.
     NAL call no. QR360.A1J6
     Descriptors: prion, chronic wasting disease, deer, nerves as routes of infection, analysis of various tissues.

223. Sihvonen L.; Tapiovaara H. BSE taudin kartoitus, nopea diagnostiikka ja ennaltaehkaisy. [Surveys, diagnosis and prevention of BSE.] Suomen-Elainlaakarilehti. 2001. 107: 1, 27-30; 8 ref. ISSN: 0039-5501. In Finnish with an English summary.
     NAL call no. 41.8 N813
     Descriptors: beef cattle, BSE, bovine spongiform encephalopathy, diagnosis, disease prevention, surveys, public health concerns, Finland, monitoring program, diagnostic tests.

224. Simon, Ortwin; Kreuzer, Michael. BSE and its consequences for the nutrition of farm animals. European Journal of Lipid Science and Technology. September, 2001; 103 (9): 563-564. ISSN: 1438-7697
     NAL call no. TP670 F472
     Descriptors: bovine spongiform encephalopathy, nervous system disease, prion disease, farm animal nutrition, food export ban, animal feed.

225. Slenczka, Werner. Mad cow disease. Emerging Infectious Diseases. 2001; 7 (3 Supplement): 605. ISSN: 1080-6040
     NAL call no. RA648.5 E46
     Descriptors: BSE, review.

226. Shlomchik, Mark J.; Radebold, Klaus; Duclos, Nicole; Manuelidis, Laura. Neuroinvasion by a Creutzfeldt-Jakob disease agent in the absence of B cells and follicular dendritic cells. Proceedings of the National Academy of Sciences of the United States of America. July 31, 2001; 98 (16): 9289-9294. ISSN: 0027-8424
     NAL call no. 500 N21P
     Descriptors: NvCreutzfeldt-Jakob Disease, infection carrying cells, scrapie, B-cells, follicular dendritic cells, prion protein, mutant mouse model B-cell negative, route of neuroinvasion, IgG.

227. Soregaroli, C. In crescita nel 2000 i mercati lattiero-caseari Europei. [The growth of European milk and cheese markets in 2000.] Informatore Agrario. 2001, 57: 12, 31-33. ISSN: 0020-0689. In Italian.
     NAL call no. 281.8 IN32
     Descriptors: BSE, bovine spongiform encephalopathy, dairy products, consumers, demand, European Union, exports, food marketing, international trade, prices, prion diseases, subsidies, support measures, surpluses, trade agreements, World Trade Organization.

228. SoRelle, R. Contaminated feed heightens "mad cow" fears in the United States. Circulation. 2001 Feb 27. 103(8): E9015-E9016 ISSN: 1524-4539
     Descriptors: BSE, cattle feed formulations, feed processing, disease risks US.

229. Soto, Claudio; Saboria, Gabriela. Prions: Disease propagation and disease therapy by conformational transmission. Trends in Molecular Medicine. March, 2001; 7 (3): 109-114. ISSN: 1471-4914
     Descriptors: transmissible spongiform encephalopathies, prions, prion protein isoforms, protein conformation as potential pharmaceutical.

230. Stanford, K.; Stitt, J.; Kellar, J.A.; McAllister, T.A. Traceability in cattle and small ruminants in Canada. Revue Scientifique et Technique International Office of Epizootics . 2001 Aug. 20(2): 510-522 ISSN: 0253-1933
     Abstract: Traceback systems for cattle and small ruminants are of international concern after the outbreaks of bovine spongiform encephalopathy in the European Union and foot and mouth disease in the United Kingdom and South America. Implementation of a national or international identification system depends on meeting a balance between cost, reliability/durability, ease of use, data transfer speed, protection from fraud, avoidance of entry into the food chain and animal welfare issues. As of 1 January 2001, Canada has instituted a national identification programme for cattle, which will have annual operating and administrative costs of Can $0.20 per head, excluding ear tags. The system will provide herd of origin traceback and individual animal identification by ear tags for all beef cattle. A number of identification technologies are available that would have advantages over visual tags, but these are currently too costly without government support (electronic identification, deoxyribonucleic acid [DNA] fingerprinting), too slow (DNA fingerprinting) or have not been tested sufficiently (retinal imaging) to warrant mandatory inclusion in a national traceback/identification system.
     NAL call no. SF781.R4
     Descriptors: BSE, foot and mouth disease, Canada, beef cattle, small ruminants, UK, South America, national identification system, ear tags.

231. Stellungnahme der Gesellschaft fur Tierzuchtwissenschaft (GfT) und der Deutschen Gesellschaft fur Zuchtungskunde (DGfZ) zur BSE-Krise. [Opinions of the Society for Animal Breeding (GfT) and the German Society for Breeding Science (DGfZ) with regard to the BSE crisis.] Zuchtungskunde. 2001, 73: 2, 81-84. ISSN: 0044-5401. In German.
     NAL call no. 49 Z8
     Descriptors: BSE, bovine spongiform encephalopathy, cattle, prion diseases, genotype databank, live sampling, tissue and blood samples, herds.

232. Stockdale, T. A biochemical theory to explain the cause of bovine spongiform encephalopathy and other encephalopathies. Medical Hypotheses 2001 Jun. 56(6): 608-616 ISSN: 0306-9877
     Abstract: The purpose of this study is to present a hypothesis to explain the aetiology of bovine spongiform encephalopathy (BSE) which is more credible than any at present available, and to increase its credibility by varying the hypothesis to supply explanations for Alzheimer's disease, Parkinson's disease and certain other conditions. The method used has been to utilize material from biochemical textbooks and similar sources. It has been concluded that BSE is caused by the failure to synthesize sufficient cyclic guanosine monophosphate (cGMP), with the result that neurons die because they are no longer able to prevent the entry of toxic quantities of calcium ions into their cytoplasm. Several causes for the failure to synthesize sufficient cGMP have been identified; these involve selenium and folate deficiencies, and problems with the availability of nicotinamide adenosine dinucleotide (NAD). It is proposed that BSE is initiated by a combination of selenium deficiency and the destruction of NAD by a bacterial toxin of the same type as causes cholera, that folate deficiency is the predominant cause of Alzheimer's disease, and that the failure to synthesize sufficient tetrahydrobiopterin and cGMP from guanosine triphosphate results in Parkinson's disease.
     Descriptors: etiology, BSE, cGMP synthesis, selemium and folate deficiencies, destruction of NAD, bacterial toxins.

233. Supattapone,S.; Wille, H.; Uyechi, L.; Safar, J.; Tremblay, P.; Szoka, F.C.; Cohen, F. E.; Prusiner, S.B.; Scott, M.R. Branched polyamines cure prion-infected neuroblastoma cells. Journal of Virology. 2001 Apr. 75(7): 3453-3461 ISSN: 0022-538X
     Abstract: Branched polyamines, including polyamidoamine and polypropyleneimine (PPI) dendrimers, are able to purge PrP(Sc), the disease-causing isoform of the prion protein, from scrapie-infected neuroblastoma (ScN2a) cells in culture (S. Supattapone, H.-O. B. Nguyen, F. E. Cohen, S. B. Prusiner, and M. R. Scott, Proc. Natl. Acad. Sci. USA 96:14529-14534, 1999). We now demonstrate that exposure of ScN2a cells to 3 microg of PPI generation 4.0/ml for 4 weeks not only reduced PrP(Sc) to a level undetectable by Western blot but also eradicated prion infectivity as determined by a bioassay in mice. Exposure of purified RML prions to branched polyamines in vitro disaggregated the prion rods, reduced the beta-sheet content of PrP 27-30, and rendered PrP 27-30 susceptible to proteolysis. The susceptibility of PrP(Sc) to proteolytic digestion induced by branched polyamines in vitro was strain dependent. Notably, PrP(Sc) from bovine spongiform encephalopathy-infected brain was susceptible to PPI-mediated denaturation in vitro, whereas PrP(Sc) from natural sheep scrapie-infected brain was resistant. Fluorescein-labeled PPI accumulated specifically in lysosomes, suggesting that branched polyamines act within this acidic compartment to mediate PrP(Sc) clearance. Branched polyamines are the first class of compounds shown to cure prion infection in living cells and may prove useful as therapeutic, disinfecting, and strain-typing reagents for prion diseases.
     NAL call no. QR360 J6
     Descriptors: in vitro testing, polyamidoamine, polypropyleneimine, scrapie infected neuroblastoma cells, PrP isoforms, proteolysis susceptibility, BSE infected, possible therapeutic use.

234. Suarez Fernandez, G. Verdad e incertidumbre actual en torno al mal de las vacas locas. [The truth and present uncertainty about mad cow disease] Anales de la Real Academia Nacional de Medicina (Madr.). 2001. 118(1): 189-206; discussion 206-215. ISSN: 0034-0634. In Spanish.
     Abstract: A historical review is made about spongiform encephalopathies which affect both animals and man. This is the base for an epidemiological and predictive analysis of these type of diseases, especially bovine spongiform encephalopathy (BSE) as a present health problem. The scientific certainties or truths, such as the prion theory (PrPc-PrPsc), the low natural infectivity of these group of diseases, the high dose of prions necessary to produce the experimental disease, the species barrier or specificity, the individual susceptibility due to genetic traits, and the low transmission efficiency by the oral route, compared to the parenteral route, agree with the epidemiological observations of human cases of the variant of the Creutzfeldt-Jakob disease (vCJD), which is 0.1 cases per million inhabitants and year. The present and future prediction of BSE should not be alarmist, taking into account the certainties that we know.
     Descriptors: BSE, spongiform encephalopathies, various theories of cause, transmission, epidemiology.

235. Tacke, V. BSE as an organizational construction: a case study on the globalization of risk. British Journal of Sociology, 2001 Jun. 52(2): 293-312 ISSN: 0007-1315
     Abstract: This article examines the BSE problem as an example of the 'globalization of risk'. In order to determine whether the 'globalization of risk' is a social construction depending on the context, the paper emphasizes the particular role of organizations. It makes an empirical comparison of the BSE-related risk-constructions of five business associations in the German meat industry sector. The results show that the associations construct the risk in close relation to their horizons of globalization, thereby reflecting provision problems, which the companies they are representing face. While the main organizational domains in the sector tried to cope with the risk problem by different means of local market 'closure', one association, founded in reaction to the BSE problem, took over a 'reflexive' role with regard to the emerging risk communication on BSE in Germany.
     Descriptors: BSE, risk communications, Germany.

236. Tagaya, M. BSE fostered by cosinesss and lack of independent advice. Nature. 2001 Nov 8. 414(6860): 147 ISSN: 0028-0836
     NAL call no. 472 N21
     Descriptors: Bovine spongiform encephalopathy, prevention and control, cattle, communication, Japan, government, politics, risk assessment.

237. Taylor, D.M.; Hester, R.E. (ed.); Harrison, R.M. Mad cows, demented humans and food. Food safety and food quality. 2001, 79-93. Published by Royal Society of Chemistry; Cambridge; UK ISBN: 0-85404-270-9
     NAL call no. TX531 F666 2001
     Descriptors: BSE, bovine spongiform encephalopathy; NvCreutzfeldt Jakob disease; transmissible spongiform encephalopathies, foodborne diseases, public health concerns, scrapie, wasting

238. Taylor, D.M.; Fernie, K.; Steele, P. J.; Somerville, R. A. Relative efficiency of transmitting bovine spongiform encephalopathy to RIII mice by the oral route. Veterinary Record. 2001 Mar 17. 148(11): 345-346 ISSN: 0042-4900
     NAL call no. 41.8 V641
     Descriptors: BSE, mouse model, laboratory experiment, transmission study.

239. Taylor, D.M.; Rabenau, H.F. (ed.); Cinatl, J. (ed.); Doerr, H.W. Resistance of transmissible spongiform encephalopathy agents to decontamination. Prions: a challenge for science, medicine and public health-system. 2001, 58-67; 48 ref. Published by: S. Karger AG; Basel; Switzerland. ISBN: 3-8055-7124-0
     NAL call no. QR1 C66 v. 7
     Descriptors: BSE, bovine spongiform encephalopathy, NvCreutzfeldt Jakob disease, decontamination, drugs, heat inactivation, prions, scrapie, transmissible spongiform encephalopathies.

240. Tegtmeier, C.; Agerholm, J.S.; Bille Hansen, V.; Schaap, P.K.; Ryder, S. First confirmed native case of bovine spongiform encephalopathy in Denmark. Vet Rec. London : The British Veterinary Association. Jan 13, 2001. v. 148 (2) p. 51-52. ISSN: 0042-4900
     NAL call no. 41.8 V641
     Descriptors: dairy cows, bovine spongiform encephalopathy, clinical aspects, brain histopathology, young-animals, case reports, Denmark.

241. Tella, J. L. Action is needed now, or BSE crisis could wipe out endangered birds of prey. Nature. 2001 Mar 22. 410(6827): 408 ISSN: 0028-0836
     NAL call no. 472 N21
     Descriptors: BSE, wildbirds, contaminated carrion, food chain concerns.

242. Terry, L.A.; Marsh, S.A.; Ryder, S.J.; Hawkins, S.A.C; Wells, G.A.H.; Spencer, Y. I. Detection of disease-specific PrP by immunohistochemistry in the distal ileum of cattle orally challenged with the BSE agent. Research in Veterinary Science. April, 2001; 70 (Supplement A): 24. ISSN: 0034-5288. 55th Annual Conference on Current Topics in Veterinary Science, Scarborough, England, UK, April 09-12, 2001
     NAL call no. 41.8 R312
     Descriptors: PrP, diagnosis, detection methods, experimental infection, cattle, intestinal tissue.

243. Thompson, C. In search of a cure for CJD. Nature 2001 vol. 409, no. 6821, pp. 660-661. ISSN: 0028-0836.
     NAL call no.  472 N21
     Descriptors: Creutzfeldt-Jakob-disease; bovine spongiform encephalopathy, therapeutic agents, prion diseases, cattle disease incidence, human exposures.

244. Tranulis, M. A.; Espenes, A.; Comincini, S.; Skretting, G.; Harbitz, I. The PrP-like protein Doppel gene in sheep and cattle: cDNA sequence and expression. Mammalian Genome. 2001 May. 12(5): 376-379 ISSN: 0938-8990
     Abstract: cDNAs encoding the ovine and bovine prion protein-like protein Doppel (Dpl) have been cloned. Sequencing revealed cDNAs of 2.85 and 3.31 kb from ovine and bovine testicular tissue, in accordance with observations of single transcripts of 3.2 and 3.6 kb on Northern blots. Sequence alignments showed a very high degree of identity between the sheep and cattle Dpl cDNAs, except for a 0.4-kb stretch in the bovine 3' untranslated region and the terminal 3' end of the sequences. The expression pattern of the Dpl gene (Prnd) in adult tissues from both species was compared by Northern blot and RT-PCR analyses. The Prnd gene was expressed strongly in testicular tissue, while low levels of expression were seen in other tissues. The open reading frame of the ovine and bovine sequences encodes a 178-amino acid protein with 95% sequence identity between the two species. Predicted structural features are in close agreement with previous reports for mouse, human, and rat Dpl.
     NAL call no. QL738.5.M359
     Descriptors: Doppel gene, sheep, cattle, genetics, sequence identity, species comparison.

245. Troeger, K. Alternative Methoden stehen zur Wahl: mehr Sicherheit bei kritischen Prozessstufen des Schlachtens und Zerlegens. [The choice of alternative methods: more security during critical process phases of slaughter and cutting up.] Fleischwirtschaft. 2001, 81: 4, 62-64; 3 ref. ISSN: 0015-363X. In German.
     NAL call no. 280.38 F62
     Descriptors: slaughter processes, abattoirs, disease control measures, bovine spongiform encephalopathy; cattle, prion diseases, risk assessment.

246. UK government has "no idea" of scale of BSE export risk. Agra Europe. (Brit. edition.) 2001, No. 1937, EP-1-EP-3. ISSN: 0002-1024
     NAL call no. 286.8 AG3AE
     Descriptors: contaminated animal protein in feeds, beef cattle, BSE, bovine spongiform encephalopathy, UK, errors of exports records, international trade, meat and bone meal, human and animal health risks in third world countries.

247. United States. Foreign Agricultural Service. Dairy, Livestock, and Poultry Division. Bovine spongiform encephalopathy (BSE). [Washington, D.C.?] : U.S. Dept. of Agriculture, Foreign Agricultural Service, Dairy, Livestock and Poultry Division, [2001?-]
     Abstract: Provides sources of information on the effects on trade of the current BSE crisis in the European Union.
     NAL call no. aSF967.S63B6846 2001
     Descriptors: Bovine spongiform encephalopathy, European Union countries, computer network resources, cattle, virus, diseases, economic aspects.

248. Urlings, H.A.; van Zijderveld, F.G. Active surveillance of BSE in cattle in The Netherlands. The Veterinary Quarterly. 2001 Jul. 23(3): 134-138 ISSN: 0165-2176
     Abstract: Since January 2, 2001 a large-scale active surveillance programme for BSE started in the Netherlands in addition to the passive surveillance programme of cattle with clinical symptoms compatible with BSE. Based on decisions of the Council of European Ministers of Agriculture, the European Union launched an active surveillance system for BSE in cattle of 30 months and older. Until April 1, more than 100,000 head of cattle were tested in this scheme, including all cattle slaughtered and a large part of the cattle that died on the farm. Four animals were found positive in the active surveillance system and one cow from the passive surveillance tested positive for BSE during the first three months.
     NAL call no. SF601 V46
     Descriptors: surveillance program, BSE, 100,000 cattle tested, slaughter, on-the-farm deaths, Netherlands.

249. Urlings, B.; Bianchi, A. BSE-testen in Nederland. [BSE testing in the Netherlands]. Tijdschrift voor Diergeneeskunde. 2001 Jan 15. 126(2): 46-49 ISSN: 0040-7453. In Dutch.
     NAL call no. 41.8 T431
     Descriptors: bovine spongiform encephalopathy, incidence of disease.

250. Urlings, B. BSE-testen op 17.000 runderen negatief. [BSE tests are negative in 17,000 cows]. Tijdschrift-voor-Diergeneeskunde. 2001 Feb 1. 126(3): 72 ISSN: 0040-7453. In Dutch.
     NAL call no. 41.8 T431
     Descriptors: bovine spongiform encephalopathy, cattle, testing program results, The Netherlands.

251. van Wuijckhuise, L.; Vellema, P.; Terbijhe, R.J. BSE: klinische diagnostiek en veldervaringen. [BSE: clinical diagnosis and field experience.] Tijdschrift voor Diergeneeskunde. 2001 Apr 15. 126(8): 279-281 ISSN: 0040-7453. In Dutch.
     Abstract: The clinical diagnosis 'suspected of having BSE' is difficult. Cows older than 2.5 years, presented to the private veterinary practitioner with disturbed locomotion present for more than 14 days and with no detectable cause, should be investigated with suspicion. Questions on changes in behaviour of the animal and during the examination attention on exaggerated responses to handling and sound can give additional information. Notification of suspected cases and gained field experiences are discussed.
     NAL call no. 41.8 T431
     Descriptors: clinical diagnosis, cattle, behavior changes, exaggerated responses to handling and sound

252. Vega, Ana; Ruiz-Ponte, Clara; Carracedo, Angel; Barros, Francisco. Rapid genotyping of the M129V polymorphism of prion protein using real-time fluorescent PCR. Clinical Chemistry. October, 2001; 47 (10): 1874-1875. ISSN: 0009-9147
     NAL call no. 396.8 C61
     Descriptors: prion proteins, BSE, genotyping, diagnostic.

253. Venters, George A. New variant Creutzfeldt-Jakob disease: The epidemic that never was. BMJ. 13 October, 2001; 323 (7317): 858-861. ISSN: 0959-8138
     Descriptors: NvCreutzfeldt-Jakob Disease, epidemiology, etiology, public health concerns.

254. Ventura, Marco; Rossi, Roberto. Il caso mucca pazza: follie di uomini e di animali. [The case of mad cow: follies of humans and animals.] Roma : Phoenix, 2001. 150 p. In Italian. ISBN: 8886732511
     NAL call no. QR201.B74 V46 2001
     Descriptors: bovine spongiform encephalopathy, epidemic issues, human risks, cattle, governmental policies and responses.

255. von Holst, Christoph; Unglaub, Wolfgang; Anklam, Elke. Post process product control of rendering plant sterilization conditions by ELISA. Journal of AOAC International. November-December, 2001; 84 (6): 1793-1798. ISSN: 1060-3271.
     NAL call no. S583 A7
     Descriptors: laboratory autoclave, commercial rendering plant, R-value, enzyme-linked immunosorbent assay (ELISA), sterilization of animal meal, temperature, duration of processing.

256. Vossen, P. Scientific advice in support to risk management with regard to BSE. Verhandelingen Koninklijke Academie voor Geneeskunde van Belgie. 2001. 63(4): 379-403 ISSN: 0302-6469
     Abstract: The paper provides an overview of the efforts put into place by the European Commission for assessing the risks for humans, animals and the environment resulting from the BSE epidemic. The risk assessments are carried out by the Scientific Steering Committee (SSC) and its TSE/BSE ad hoc Group. They are part of the EC's scientific advisory system in support of health and consumer protection policy, which comprises another 8 scientific committees. The process from the emergence of a possible reason for concern to the submission of a legislative proposal based on a scientific opinion is outlined and the careful, step-wise process of preparing a TSE risk assessment is explained. The TSE-related assessments are mostly qualitative. An example of a possible quantitative approach is given and it is shown that appropriate data and current scientific knowledge still do not permit the preparation of fully comprehensive quantitative risk assessments. Moreover, given the many unknowns and uncertainties, it is not evident that quantitative risk assessments would automatically provide the risk managers with a more appropriate tool in support of decision making. The multi-disciplinary of the assessments is highlighted and an overview is given of the TSE-related risk assessments carried out since 1997. They cover a wide range of fields, including safe geographical sourcing of animals, infectivity inactivation by processing, human exposure and epidemiology.
     Descriptors: BSE; human, animal, environmental risks; European Commission, health and consumer policy, epidemiology, inactivation of animal products.

257. Wadman, M. Agencies face uphill battle to keep United States free of BSE. Nature. 2001 Jan 25. 409(6819): 441-442 ISSN: 0028-0836
     NAL call no. 472 N21
     Descriptors: Legislation, cattle, commerce, deer, Creutzfeldt-Jakob Syndrome, prevention and control, government agencies, human, meat, United States, United States Department of Agriculture, United States Food and Drug Administration.

258. Watts, J. Bovine spongiform encephalopathy case found in Japan. Lancet. 2001 Sep 22. 358(9286): 991 ISSN: 0140-6736
     NAL call no. 448.8 L22
     Descriptors: cattle, BSE, confirmed case, contaminated feed, UK, Japan.

259. Weber, W. Germany's Health and Agriculture ministers resign over handling of BSE. Lancet. 2001 Jan 20. 357(9251): 207 ISSN: 0140-6736
     NAL call no. 448.8 L22
     Descriptors: Germany, government policies, politics, bovine spongiform encephalopathy.

260. Weiss, Robin A. The Leeuwenhoek Lecture 2001. Animal origins of human infectious disease. Royal Society Philosophical Transactions Biological Sciences 356(1410), 29 June, 2001: 957-977.
     NAL call no. 501 L84B
     Descriptors: microbial diseases, prion diseases, NvCreutzfeldt-Jakob disease, BSE, other diseases, zoonoses.

261. Weissenbacher, Manfred. Rinderwahnsinn: die Seuche Europas. [The case of mad cow: follies of humans and animals.] Wien : Bohlau, c2001. 160 p., ill. In German.
     NAL call no. SF967 S63 W44 2001
     Descriptors: bovine spongiform encephalopathy.

262. Wenz, B.; Oesch, B.; Horst, M. Analysis of the risk of transmitting bovine spongiform encephalopathy through bone grafts derived from bovine bone. Biomaterials. 2001 June. 22(12): 1599-1606 ISSN: 0142-9612
     Abstract: Bone substitutes of bovine origin are widely used for treatment of bone defects in dental and orthopedic surgery. Due to the occurrence of BSE and the new variant of Creutzfeldt Jakob Disease risks of transmitting diseases through the use of such materials need to be carefully evaluated. Risk analysis can either be based on theoretical assessments or experimental evidence. Here we present a comparative study on two bovine bone substitutes (Bio-Oss and Osteograf/N) which is based on theoretical values. Furthermore, for one of these materials, i.e. Bio-Oss, the prion inactivation capacity of one of the production steps was experimentally evaluated. Theoretical and experimental data indicate that the use of these materials does not carry a risk of transmitting BSE to patients.
     NAL call no. R857.M3B48
     Descriptors: bovine based bone products and grafts, BSE, NvCreutzfeldt-Jakob Disease, transmission risks to humans.

263. Weyandt, R.G. Detection of BSE-risk materials. Fresenius' Journal of Analytical Chemistry. 2001 Oct. 371(4): 574-575. ISSN: 0937-0633
     NAL call no. QD71.F7
     Descriptors: BSE, contaminated bovine based products, diagnostic procedures.

264. Williams, N. Chasing the BSE agent. Current Biology CB 2001 May 15. 11(10): R377 ISSN: 0960-9822
     NAL call no. QH301.C85
     Descriptors: bovine spongiform encephalopathy, diagnosis, risks, cause.

265. Winklhofer, K. F.; Hartl, F. U.; Tatzelt, J. A sensitive filter retention assay for the detection of PrP(Sc) and the screening of anti-prion compounds. FEBS Letters. 2001 Aug 10. 503(1): 41-45 ISSN: 0014-5793
     Abstract: A hallmark of prion diseases is the accumulation of an abnormally folded prion protein, denoted PrP(Sc). Here we describe a new and highly sensitive method for the detection of PrP(Sc) in brain and other tissue samples that utilizes both PrP(Sc) diagnostic criteria in combination; protease resistance and aggregation. Upon filtration of tissue extracts derived from scrapie- or bovine spongiform encephalopathy-infected animals, PrP(Sc) is retained and detected on the membranes. Laborious steps such as SDS-PAGE and Western blotting are avoided with concomitant gain in sensitivity and reliability. The new procedure also proved useful in a screen for anti-prion compounds in a scrapie-infected cell culture model.
     NAL call no. QD415.F4
     Descriptors: prion proteins, PrP(Sc), scrapie infected cell culture model, screening method, anti-prion compounds.

266. Wolferstan, F. A preliminary report on a study of BSE in cattle in relation to farm husbandry: Its possible relevance to vCJD and infertility problems in humans. Journal of Nutritional and Environmental Medicine, Abingdon. September, 2001; 11 (3): 205-216. ISSN: 1359-0847
     NAL call no. RM214.J68
     Descriptors: BSE, effects of husbandry, survey, 53 farms, dairy cattle, organophosphate exposure in dams, copper deficiency, grain storage chemicals, etiology, NvCreutzfeldt-Jakob Disease.

267. Woodbury, Murray R. Chronic wasting disease - the North American situation. Proceedings of a Deer Course for Veterinarians 18, May, 2001: 47-51.
     Descriptors: chronic wasting disease; deer, elk, North America.

268. Yam, P. Mad cow's human toll. Scientific American 2001 May. 284(5): 12-13 ISSN: 0036-8733
     NAL call no. 470 Sci25
     Descriptors: BSE, bovine spongiform encephalopathy, prion diseases, NvCreutzfeldt-Jakob disease, transmissibility, human health risks.

269. Yedidia,Y.; Horonchik, L.; Tzaban, S.; Yanai, A.; Taraboulos, A. Proteasomes and ubiquitin are involved in the turnover of the wild-type prion protein. EMBO Journal 2001 Oct 1. 20(19): 5383-5391
     Abstract: Prion diseases propagate by converting a normal glycoprotein of the host, PrP(C), into a pathogenic "prion" conformation. Several misfolding mutants of PrP(C) are degraded through the ER-associated degradation (ERAD)-proteasome pathway. In their infectious form, prion diseases such as bovine spongiform encephalopathy involve PrP(C) of wild-type sequence. In contrast to mutant PrP, wild-type PrP(C) was hitherto thought to be stable in the ER and thus immune to ERAD. Using proteasome inhibitors, we now show that approximately 10% of nascent PrP(C) molecules are diverted into the ERAD pathway. Cells incubated with N-acetyl-leucinal-leucinal-norleucinal (ALLN), lactacystin or MG132 accumulated both detergent-soluble and insoluble PrP species. The insoluble fraction included an unglycosylated 26 kDa PrP species with a protease-resistant core, and a M(r) "ladder" that contained ubiquitylated PrP. Our results show for the first time that wild-type PrP(C) molecules are subjected to ERAD, in the course of which they are dislocated into the cytosol and ubiquitylated. The presence of wild-type PrP molecules in the cytosol may have potential pathogenic implications.
     NAL call no. QH506 E46
     Descriptors: prions, proteasome inhibitors, ERAD, cytosol and ubiquitylated, pathogenic implications.

270. Yuen, K.Y. Emerging infectious diseases in Hong Kong. Hong Kong Medical Journal. September, 2001; 7 (3): 224-226. In English. ISSN: 1024-2708
     Descriptors: BSE, Hong Kong, zoonotic diseases.

271. Zinsstag, J.; Weiss, M.G. Livestock diseases and human health. Science. 2001 Oct 19. 294(5542): 477 ISSN: 0036-8075
     NAL call no. 470 Sci2
     Descriptors: food safety, zoonotic diseases, human health risks, BSE, E. coli.

272. Zipf, M. Change of course in consumer protection. Deutschland. 2001, No. 2, 6-10. ISSN: 0340-5788
     Descriptors: agricultural and food policy; BSE, bovine spongiform encephalopathy; public health concerns, food-production; food safety; Germany.

273. Zmudzinski, J.F.; Polak, M.P. BSE -- mity i fakty. [BSE -- myths and facts.] Zycie Weterynaryjne. 2001, 76: 3, 131-137; 27 ref. ISSN: 0137-6810. In Polish.
     NAL call no. SF604.Z9
     Descriptors: BSE, bovine spongiform encephalopathy, disease prevention, epidemiology, meat hygiene, prion disease pathology, public health risks.

274. Zusammenfassung der wesentlichen Aussagen und Vorschlaege im Zusammenhang mit der BSE vom Robert-Koch-und Paul-Ehrlich-Institut. [Summary of main statements and proposals by the Robert Koch and Paul Ehrlich institutes, Germany, concerning bovine spongiform encephalopathy (BSE).] Tieraerztliche Umschau. 1 December, 2001; 56 (12): 630-632. In German. ISSN: 0049-3864.
     NAL call no. 41.8 T445
     Descriptors: BSE, Germany, Robert Koch Institute, Paul Elrlich Institute, statements and proposals.

275. Zuppiroli, M.; Mancini, M.C. Mais in salute, ma con l'interrogativo ogm. [Maize for health, but a question mark for the genetically modified type.] Informatore Agrario. 2001, 57: 7, 47-50. ISSN: 0020-0689. In Italian.
     NAL call no. 281.8 IN32
     Descriptors: BSE, bovine spongiform encephalopathy, crop management, feeds, maize, corn marketing, transgenic crop plants, possible impact on BSE, cattle.

2000

1. Abbasi, K. BSE inquiry plays down errors. BMJ Clinical Research edition.. 2000 Nov 4; 321(7269): 1097 ISSN: 0959-8138
   NAL call no. R31 B55
   Descriptors: BSE, bovine spongiform encephalopathy, Nv Creutzfeldt-Jacob disease, government enquiry, UK Ministry, cattle, human health risks.

2. Abbott, A; Schiermeier, Q. Germany rues 'complacency' over BSE testing strategy. Nature. Nov. 30, 2000. v. 408 (6812) p. 506 ISSN: 0028-0836.
   NAL call no. 472 N21
   Descriptors: bovine spongiform encephalopathy, government, screening, cattle, cattle disease, food safety, Creutzfeldt Jakob disease, Germany.

3. Abiola, O. O.; Anderton, B. H.; Plomin, R.; Whatley, S. A. Do female sex steroids affect transmission of TSEs? Journal of Endocrinology. November, 2000. v. 167 (Supplement) p. P85. 191st Meeting of the Society for Endocrinology. London, England, UK. November 20-21, 2000. Poster abstract.
   NAL call no. 448.8 J8293
   Descriptors: mouse models, transmissible spongiform encephalopathy, prion disease, scrapie, effect of estrogens.

4. Acting on the lessons of BSE. Vet rec. London : The British Veterinary Association. Nov 4, 2000. v. 147 (19) p. 525. ISSN: 0042-4900
   NAL call no. 41.8 V641
   Descriptors: bovine spongiform encephalopathy, food safety guidelines.

5. Adjou, K. T.; Seman, M.; Brugere-Picoux, J. Diagnostic methods for subacute transmissible spongiform encephalopathies. [Methodes de diagnostic des encephalopathies subaigues spongiformes transmissibles.] Bulletin Bimestriel de la Societe Veterinaire Pratique de France. 2000. v. 84 (3) p. 163-173
   Descriptors: reviews, diagnostic methods, prion diseases, transmissible spongiform encephalopathies, Creutzfeldt-Jakob Disease, bovine spongiform encephalopathy, public health, prion proteins, cattle, man.

6. Aguzzi, A. Prion diseases, blood and the immune system: concerns and reality. Haematologica. Jan 2000. 85(1): 3-10. ISSN: 0390-6078.
   Abstract: There is a great amount of uncertainty about the nature of the agent which causes spongiform encephalopathies. In recent years the occurrence of bovine spongiform encephalopathy and of new variant-Creutzfeldt Jakob disease, has raised concerns that prions may, under certain circumstances, contaminate the blood supply. This review article illustrates the problems with which research in this field is fraught, and presents some of the arguments which are controversially discussed in the field.
   Descriptors: BSE, NvCJD, public health risks, review, discussion of issues.

7. Aldhous, P. CJD survey offers Britain a glimmer of hope. Nature. May 4, 2000. v. 405 (6782) p. 7 ISSN: 0028-0836
   NAL call no. 472 N21
   Descriptors: BSE, health survey, Creutzfeldt Jakob disease, epidemiology immunohistochemistry, appendix, tonsil, epidemic, bovine spongiform encephalopathy, UK

8. Aldhous, P. Inquiry blames missed warnings for scale of Britain's BSE crisis. Nature. 2000 Nov 2; 408(6808): 3-5 ISSN: 0028-0836
   NAL call no. 472 N21
   Descriptors: BSE, bovine spongiform encephalopathy, UK, governnment response, cattle prion disease incidence, policies.

9. Aldhous, P. Abbott, A. Neurodegeneration. Battling the killer proteins. Nature. 2000 Dec 21-28; 408(6815): 902-3. ISSN: 0028-0836
   NAL call no. 472 N21
   Descriptors: prion disease, prion proteins, BSE, CJD, transmissible spongiform encephalopathies, humans, animal.

10. Alter, M. How is Creutzfeldt-Jakob disease acquired? Neuroepidemiology. Mar-Apr 2000. 19(2): 55-61. ISSN: 0251-5350.
    Abstract: Creutzfeldt-Jakob disease (CJD) is one of several related disorders collectively called prion diseases. These disorders affect man and animals and are now known to be caused by the abnormal configuration of a naturally occurring protein, PrP(c). By mechanisms still not well understood, this natural protein is converted into a pathologic variant, PrP(sc). The disease is 'acquired' spontaneously perhaps by posttranslational conversion of a PrP(c) into a PrP(sc) population. This sporadic form of CJD has been reported worldwide with a frequency of 1/million. Other modes of acquisition include the following: ingestion of brain tissue from deceased victims through ritual cannibalism at burial ceremonies formerly (and no longer) practiced by New Guinea Highlanders; iatrogenically, through corneal transplants from infected donors, inoculation of human growth hormone and gonadotropin prepared from infected human pituitary glands; from inadequately sterilized depth electrodes introduced neurosurgically into the brain during workups of patients with epilepsy, and applications of infected dura mater in neurosurgical procedures. Most recently, an infected bovine source (bovine spongiform encephalopathy) has been implicated and produces a new variant of CJD. Clusters of CJD in families in some populations have been recognized which are inherited in Mendelian fashion. These clusters are related to mutations of the PRNP gene in specific codons (e.g. codon 200). Homozygosity for these mutations increases the chances of manifesting the disease. Other potential methods of acquisition, such as by blood transfusion, surgical sutures, tonometers, consumption of hog brain or other organs and tissue, remain unproven.
    Descriptors: modes of acquisition, iatrogenic transmission, BSE, NvCJD, PrPsc, contaminated foods.

11. Amasino, C. F.; Amasino, M. F.; Zaccardi, E. M. Immunological investigation of the presence of bovine proteic components in nutritional, cosmetic and medicinal products for human use: its relation with zoonoses. [Investigacion inmunologica de la presencia de componentes proteicos bovinos en productos alimenticios, cosmeticos y medicinales de uso humano: su relacion con las zoonosis.] Veterinaria Argentina. 2000. v. 17 (164) p. 275-279
    Abstract: An immunodiffusion test was used to detect bovine proteic antigens in 13 foods (including beef, dried milk, and orange juice), 6 cosmetics, and 6 drugs, sold in Argentina. Products with these proteins are potential transmitters of zoonotic diseases (including BSE). Bovine proteic antigens were found in 9 products.
    Descriptors: zoonoses, antigens, drugs, dried milk, beef, fruit juices, methodology, detection, bovine spongiform encephalopathy, animal diseases, public health, antigen testing, man, Argentina

12. Anderson, W. A. The future relationship between the media, the food industry and the consumer. British Medical Bulletin. 2000. v. 56 (1) p. 254-268
    Abstract: The relationship between the media, the food industry and the consumer is probably at its lowest point as we start the new millennium. The frequency of food scares appears to be increasing and news reports sometimes seem both sensational and polarised. High profile issues like the development of bovine spongiform encephalopathy in the UK and the dioxin contamination of poultry products in Belgium have undermined consumer confidence in the food industry. The recent genetically modified foods' debate has served to demonstrate the gulf that has grown between the food industry, food safety experts and the public. This is a rift that has been exploited by environmental pressure groups and fuelled by the media. This paper examines some of the underlying causes of the current air of mistrust that seems to exist between the media, the food industry and the consumer. Also, by examining the projected trends in these root causes, it draws some conclusions for the future relationship between the parties involved and suggests some changes that may improve the present situation.
    NAL call no. 448.2 B772
    Descriptors: BSE, bovine spongiform encephalopathy, prion diseases, dioxin, food safety, consumer issues, Belgium, United Kingdom

13. Andesen, P. Bovine spongiform encephalopathy. [Bovin spongiform encephalophati.] Dansk Veterinaertidsskrift. 2000. v. 83 (7) p. 18-19, 10 refs.
    NAL call no. 41.9 D23
    Descriptors: bovine spongiform encephalopathy, BSE, pathogenesis, symptoms, nervous system diseases, prion diseases, cattle.

14. Ansfield, M.; Reaney, S.D.; Jackman, R. Performance assessment and validation of a sensitive immunoassay for detection of ruminant and porcine heat stable proteins in compound animal feedstuffs. Food and Agricultural Immunology. 2000, 12: 4, 285-297; 6 ref. ISSN: 0954-0105
    NAL call no. QR183.6.F66
    Descriptors: Sandwich ELISA, feeds components analysis, analytical methods, immunological techniques, contamination, livestock, bovine spongiform encephalopathy, disease control, meat and bone meal, detection methods.

15. Ansfield, M.; Reaney, S.D.; Jackman, R. Production of a sensitive immunoassay for detection of ruminant and porcine proteins, heated to > 130░C at 2.7 bar, in compound animal feedstuffs. Food and Agricultural Immunology. 2000, 12: 4, 273-284; 5 ref. ISSN: 0954-0105
    NAL call no. QR183.6.F66
    Descriptors: ELISA, analytical methods, feeds formulations, feed component analysis, identification of source animal proteins, immunological techniques, contamination, BSE, bovine spongiform encephalopathy, disease control measures, meat and bone meal.

16. Antloga, K.; Meszaros, J.; Malchesky, P.S.; McDonnell, G.E. Prion disease and medical devices. ASAIO Journal. 2000. v. 46 (6) p. S69-S72
    Descriptors: prions, PrP, transmissible spongiform encephalopathies, iatrogenic transmission, peracetic acid based sterilant, recommended clinical practices, sterilants.

17. Ashraf, H. BSE inquiry uncovers "a peculiarly British disaster". Lancet. 2000 Nov 4; 356(9241): 1579-80 ISSN: 0140-6736
    NAL call no. 448.8 L22
    Descriptors: BSE, bovine spongiform encephalopathy, Nv Creutzfeldt-Jacop disease, UK government, Spongiform Encephalopathy Advisory Committee, safety of beef products, role of scientific advisory committees in policy, interdepartmental rivalry, Chief Medical Officer, Chief Veterinary Officer, compensation and care for NvCJD patients, scrapie, cattle feed bans, sheep, research initiatives, review of the epidemic, intensive farming systems, trade, Lord Phillips report of public inquiry

18. Austin, A. R.; Bergamini, P. F. The clinical manifestations of bovine spongiform encephalopathy (BSE). In the Congresso nazionale Stresa (VB) 5-6-7 maggio 2000 e Giornate buiatriche "La figura del medico-veterinario nella filiera della produzione lattiero-casearia regionale" Asiago (VI) 29 maggio 1999, e "Vitello a carne bianca" Ozzano Emilia (BO) 20 novembre 1999. Atti della Societa Italiana di Buiatria. 2000. v. 32 p. 3-7, 15 refs.
    Descriptors: bovine spongiform encephalopathy, disease description, spongiform encephalopathy, symptoms, diagnosis, cattle, UK.

19. Baines, P.R.; Harris, P. Kite flying: the role of marketing in the post-BSE British beef export industry. British Food Journal. Bradford : MCB University Press. 2000. v. 102 (5/6) p. 454-464. ISSN: 0007-070X. In the special issue: Marketing issues in the new millennium examined by the case study method / edited by C. Vignali.
    Abstract: Outlines the role of the Meat Livestock Commission in dealing with the BSE/CJD crisis in the UK meat industry. It covers the re-launch of British beef, the history of the BSE crisis, the decline of the export market for beef and the increasingly political nature of the world beef and meat markets. In addition, the article assesses the impact of supermarkets, government and environmental concerns and the development of this key industry. The issue of reputation alongside natural products is considered and the complexities of a fragmented market. Suggests that there is no quick and easy fix to the re-establishment of UK roast beef as a premier brand.
    NAL call no. 389.8 B77
    Descriptors: beef, food industry, international trade, exports, food marketing, bovine spongiform encephalopathy, commodity markets, supermarkets, government policy, food-products, consumer attitudes, UK.

20. Balter, M. Experts downplay new vCJD fears. Science. Sep. 8, 2000. v. 289 (5485) p. 1663-1666
    NAL call no. 470 SCI2
    Descriptors: food contamination, food poisoning, etiology, Salmonella, BSE, bovine spongiform encephalopathy, UK, Creutzfeldt Jakob disease, disease transmission, chicken, human, nonhuman.

21. Balter, M. Hunt for mad cow in sheep reassuring. Science. Aug. 11, 2000. v. 289 (5481) p. 849
    NAL call no. 470 SCI2
    Descriptors: BSE, bovine spongiform encephalopathy, diagnosis, etiology, sheep, Creutzfeldt Jakob disease, scrapie, United Kingdom

22. Balter, M. On the hunt for a wolf in sheep's clothing. Science. March 17, 2000. v. 287 (5460) p. 1906-1908
    NAL call no. 470 SCI2
    Descriptors: scrapie, BSE, bovine spongiform encephalopathy, NvCreutzfeldt-Jakob Disease, disease transmission risks

23. Balter, M. Tracking the human fallout from 'mad cow disease'. Science (Washington). 2000. v. 289 (5484) p. 1452-1454 ISSN: 0036-8075
    NAL call no. 470 SC12
    Descriptors: Creutzfeldt-Jakob Disease, bovine spongiform encephalopathy, prion diseases, disease surveys, epidemiology, UK.

24. Banati, D. The development of European food law - from the 'principle of the free movement of goods' to the 'precautionary principle'. Part 1. [Az Europai elelmiszerjog fejlodese a "termekek szabad aramlasanak elvetol" az "elovigyazatossag elveig" I. r.] Elelmezesi Ipar. 2000. v. 54 (2) p. 46-48.
    NAL call no. 290.9 M57
    Descriptors: food safety legislation, European Union, consumer confidence, hormones, dioxins, bovine spongiform encephalopathy, BSE, free trade, genetically modified foods.

25. Barbier, M.; Joly, P.B. La securite alimentaire a l'epreuve de la crise de l'ESB: obsession du risque ou emergence d'une democratie des risques? [Food safety during the BSE crisis: obsession with risks or emergence of a risk democracy?] 7emes Rencontres autour des recherches sur les ruminants, Paris, France, 6-7 Decembre-2000. 2000, 7 (supplement), 39-44. Institut National de la Recherche Agronomique (INRA); Paris; France. In French.
    NAL call no. SF191.2 R46
    Descriptors: food safety concerns, risk assessment, bovine spongiform encephalopathy, cattle prion diseases, consumer demands and behaviour, mass media, meat and livestock industry.

26. Barnard, G.; Helmick, B.; Madden, S.; Gilbourne, C.; Patel, R. The measurement of prion protein in bovine brain tissue using differential extraction and DELFIA as a diagnostic test for BSE. Luminescence the journal of biological and chemical luminescence. Nov-Dec 2000. 15(6): 357-362. ISSN: 1522-7235.
    Abstract: A simple diagnostic test for the detection of bovine spongiform encephalopathy (BSE), based on a commercially available time-resolved fluorescence immunoassay (DELFIA) for the measurement of the normal and disease-associated isoforms of prion protein (PrP), is described. The isoforms are sequentially extracted from homogenized bovine brain tissue using two concentrations of guanidine hydrochloride. This procedure initially extracts a soluble isoform and subsequently a less soluble disease-associated aggregated isoform. Following quantification of the two fractions, the percentage of the insoluble prion becomes a measurable parameter, independent of protein concentration, clearly identifying normal from infected animals displaying clinical signs of BSE. The mean percentages of insoluble PrP in brain tissue from 60 BSE-confirmed-positive cattle and 100 cattle that had never been exposed to the disease were 52.6% (SD = 22.8) and 3.9% (SD = 1.5), respectively. The assay is sensitive, with a detection limit of less than 50 pg PrP, and is robust and precise (CVs < 10%) over the appropriate working range.
    Descriptors: BSE, diagnostic tests, cattle brains, PrP, assay sensitivity.

27. Baron, T. Transmission of bovine spongiform encephalopathy to man: current knowledge. [Transmissibilite de l'encephalopathie spongiforme bovine a l'homme: etat des connaissances actuelles.] Le Point Veterinaire. 2000. v. 31 (207) p. 189-194, 16 refs.
    NAL call no. SF602 P6
    Descriptors: bovine spongiform encephalopathy, prion diseases, disease transmission to man, Creutzfeldt-Jakob Disease, meat, zoonoses, public health risks, cattle.

28. Baron Thierry, G. M.; Madec, Jean-Yves; Calavas, Didier; Richard, Yves; Barillet, Francis Comparison of French natural scrapie isolates with bovine spongiform encephalopathy and experimental scrapie infected sheep. Neuroscience Letters. April 28, 2000. v. 284 (3) p. 175-178
    Abstract: The authors compared the glycoform pattern of the abnormal prion protein (PrPSc) detected by immunoblotting in 21 sheep with natural scrapie, (from 21 different outbreaks identified in France since 1996), with a bovine spongiform encephalopathy (BSE)-infected sheep. All the natural scrapie isolates had a higher molecular mass of the unglycosylated PrPSc than in BSE-infected sheep. In the latter case, this molecular mass appeared identical to that found in the CH 1641 experimental scrapie strain (type C pattern), whereas in natural scrapie cases it was similar to that found in the SSBP/1 experimental scrapie strains. These results suggest that all French natural scrapie isolates studied so far would belong, as SSBP/1, to the group of scrapie cases with type A electrophoretic pattern.
    NAL call no. QP351 N3
    Descriptors: glycoform pattern, abnormal prion protein (PrPSc), bovine spongiform, BSE, sheep, encephalopathy, experimental infection, CH 1641 experimental scrapie strain, immunoblotting, natural scrapie, France.

29. Bartz, J. C.; Bessen, R. A.; McKenzie, D.; Marsh, R. F.; Aiken, J. M. Adaptation and selection of prion protein strain conformations following interspecies transmission of transmissible mink encephalopathy. Journal of virology. Jun 2000. 74(12): 5542-5547. ISSN: 0022-538X.
    Abstract: Interspecies transmission of the transmissible spongiform encephalopathies (TSEs), or prion diseases, can result in the adaptation and selection of TSE strains with an expanded host range and increased virulence such as in the case of bovine spongiform encephalopathy and variant Creutzfeldt-Jakob disease. To investigate TSE strain adaptation, we serially passaged a biological clone of transmissible mink encephalopathy (TME) into Syrian golden hamsters and examined the selection of distinct strain phenotypes and conformations of the disease-specific isoform of the prion protein (PrP(Sc)). The long-incubation-period drowsy (DY) TME strain was the predominate strain, based on the presence of its strain-specific PrP(Sc) following interspecies passage. Additional serial passages in hamsters resulted in the selection of the hyper (HY) TME PrP(Sc) strain-dependent conformation and its short incubation period phenotype unless the passages were performed with a low-dose inoculum (e.g., 10(-5) dilution), in which case the DY TME clinical phenotype continued to predominate. For both TME strains, the PrP(Sc) strain pattern preceded stabilization of the TME strain phenotype. These findings demonstrate that interspecies transmission of a single cloned TSE strain resulted in adaptation of at least two strain-associated PrP(Sc) conformations that underwent selection until one type of PrP(Sc) conformation and strain phenotype became predominant. To examine TME strain selection in the absence of host adaptation, hamsters were coinfected with hamster-adapted HY and DY TME. DY TME was able to interfere with the selection of the short-incubation HY TME phenotype. Coinfection could result in the DY TME phenotype and PrP(Sc) conformation on first passage, but on subsequent passages, the disease pattern converted to HY TME. These findings indicate that during TSE strain adaptation, there is selection of a strain-specific PrP(Sc) conformation that can determine the TSE strain phenotype.
    NAL call no. QR360.J6
    Descriptors: TSE strain adaptation, strain phenotype, PrPsc changes, Syrian golden hamsters, serial passage, incubation times.

30. Behizad, M.; Curling, J.M. Comparing the safety of synthetic and biological ligands used for purification of therapeutic proteins. BioPharm. 2000. v. 13 (9) p. 42-46
    NAL call no. RM301.4 B55
    Descriptors: protein purification, disease transmission, Food and Drug Administration, safety concerns, possible contamination, adsorption, affinity chromatography, transgenic animal, bovine spongiform encephalopathy, scrapie, complex formation, cell culture, phage display, ligand binding, topical review.

31. Birmingham, K. Europe increases precautions against BSE transmission. Nature Medicine. 2000. v. 6 (12) p. 1301.
    Descriptors: BSE transmission, bovine spongiform encephalopathy, zoonotic diseases, Creutzfeldt Jakob disease, epidemiology, scrapie, meat industry, drug manufacture, regulatory mechanism, Europe, UK.

32. Blystad, Hans. Human prion diseases in Norway. Norsk Veterinaertidsskrift. 2000. v. 112 (5) p. 400-403
    Abstract: Only sporadic cases of CJD have been identified in Norway. Norway participates in a collaborative study to document the incidence CJD in Europe. All suspected cases of human prion diseases are registered in the Norwegian Surveillance System for Communicable Diseases (MSIS). In 1997 there were ten suspected cases. Seven of these were sporadic CJD as confirmed by autopsy. In 1998 only two cases of definite sporadic CJD were noted. Scientific evidence indicates that new variant CJD is the human form of bovine spongiform encephalopathy (BSE).
    NAL call no. 41.8 N81
    Descriptors: Creutzfeldt-Jakob Disease, CJD, Kuru, Sporadic CJD, familiar CJD, iatrogenic CJD and new variant CJD, relationship to bovine spongiform encephalopathy, prion diseases, Norway, Norwegian Surveillance System for Communicable Diseases (MSIS).

33. Bogaert, E. Advice on the risk of bovine spongiform encephalopathy (BSE) in humans from blood transfusion. [Advies over het risico op boviene spongiforme encefalopathie (BSE) bij mensen door transfusie van bloed van geinfecteerde personen.] Tijdschrift voor Geneeskunde. June 15, 2000. v. 56 (12) p. 901-903
    Descriptors: medical assessment, BSE, bovine spongiform encephalopathy, blood transfusion risks, pathogenesis, Creutzfeldt Jakob disease

34. Bonetta, L. US monitors TSE in livestock. Nature Medicine. 2000. v. 6 (12) p. 1301.
    Descriptors: BSE, bovine spongiform encephalopathy, transmissible spongiform encephalopathies, epidemiology, wasting syndrome, scrapie, prion disease, US.

35. Bonn, D. New predictions for total vCJD mortality lower than before. Lancet. 2000 Aug 12; 356(9229): 570 ISSN: 0140-6736
    NAL call no. 448.8 L22
    Descriptors: BSE, bovine spongiform encephalopathy, NvCreutzfeldt-Jakob Disease, public health risks, transmission to humans, projections of human mortality.

36. Bons, N.; Cohen-Solal, C.; Mestre-Frances, N. Bovine spongiform encephalopathy in zoological parks. Folia Primatologica. July-Aug. 2000. v. 71 (4) p. 254. 11th Annual Meeting of the French Language Society of Primatology. Paris, France. September 29-October 02, 1999.
    NAL call no. QL737 P9F6
    Descriptors: BSE, prion diseases, zoological parks, Microcebus murinus, risks of disease transmission, Montpellier Zoo, France.

37. Bosch, X. Spain's BSE cattle embargo causes tension at European Commission. Lancet. 2000 Nov 18; 356(9243): 1746 ISSN: 0140-6736
    NAL call no. 448.8 L22
    Descriptors: BSE, bovine spongiform encephalopathy, cattle prion disease, trade embargo on bovine products, EU, Spain.

38. Bovine spongiform encephalopathy and the safety of human diet. [Boviene spongiforme encefalopathie en de veiligheid van voedsel.] Nederlands Tijdschrift voor Geneeskunde. May 27, 2000. v. 144 (22) p. 1052-1057
    Descriptors: BSE, bovine spongiform encephalopathy, dietary intake, consumer risks, Creutzfeldt Jakob disease, cattle, food safety, genetic predisposition, topical review

39. Bradley, R. Bovine spongiform encephalopathy (BSE) and variant Creutzfeldt-Jakob Disease (vCJD). Cattle Practice. 2000. v. 8 (2) p. 151-153, 5 refs.
    NAL call no. SF961 C37
    Descriptors: New variant Creutzfeldt-Jakob Disease, bovine spongiform encephalopathy, BSE, animal health, disease control, geographical distribution, public health, animal-based feeds.

40. Bradley, R. Veterinary research at the Central Veterinary Laboratory, Weybridge, with special reference to scrapie and bovine spongiform encephalopathy. Revue Scientifique et Technique Office International des Epizooties. Dec. 2000. v. 19 (3) p. 819-830.
    NAL call no. SF781 R4
    Descriptors: veterinary medicine research, United Kingdom, history, scrapie, bovine spongiform encephalopathy, nervous system disease prion disease.

41. Branscheid, W. IMS-workshop: Meat marketing in the industrialized world. Part 2: health and food safety, quality management, care of the domestic market and the enjoyment value of meat. [IMS-Worshop: Fleischmarketing in der industrialisierten Welt.] Fleischwirtschaft. 2000. v. 80 (4) p. 86-91. Workshop summaries are included. Topics include issues of UK beef and the BSE crisis; Germany described their meat quality assurance program; the Netherlands described the IKB program, other countries discussed export concerns, brand names, advertising strategies.
    NAL call no. 280.38 F62
    Descriptors: bovine spongiform encephalopathy, BSE crisis in the UK, beef, domestic markets, quality controls, marketing channels, pork, exports, brand names, advertising strategies, marketing policy.

42. Brown, P. BSE and transmission through blood. Lancet. Sep. 16, 2000. v. 356 (9234) p. 955-956
    NAL call no. 448.8 L22
    Descriptors: BSE, bovine spongiform encephalopathy, epidemiology, NvCreutzfeldt Jakob disease, epidemiology, disease transmission, blood, UK, prion disease, scrapie, animal disease, inoculation, blood transfusion, sheep.

43. Bruce, M.E.; Brown, K.L.; Mabbott, N.A.; Farquhar, C.F.; Jeffrey, M. Follicular dendritic cells in TSE pathogenesis. Immunology Today. 2000. v. 21 (9) p. 442-446, 38 refs.
    Abstract: The pathogenesis of transmissible spongiform encephalopathies (TSEs) often includes a replication phase in lymphoid tissues before infection spreads to the central nervous system. Recent studies show that the follicular dendritic cells of the germinal centres are critical for this replication. These cells are therefore potential targets for therapy or prophylaxis in natural TSEs, such as variant Creutzfeldt-Jakob disease.
    NAL call no. QR180 I56
    Descriptors: transmissible spongiform encephalopathy, pathogenesis, spongiform encephalopathy, scrapie, bovine spongiform encephalopathy, disease models, prion diseases, Creutzfeldt-Jakob Disease, dendritic cells, mice.

44. Bruce, M.E. 'New variant' Creutzfeldt-Jakob Disease and bovine spongiform encephalopathy. Nature Medicine. 2000. v. 6 (3) p. 258-259
    Descriptors: NvCreutzfeldt-Jakob Disease, bovine spongiform encephalopathy, BSE, conventional mice, transgenic mouse model, disease transmission, incubation time

45. Brun, A.; Castilla, J.; Torres, J. M. Encefalopatias espongiformes transmisibles en animales. [Transmissible spongiform encephalopathies in animals]. Revista de neurologia. Jul 16-31, 2000. 31(2): 133-137. ISSN: 0210-0010. In Spanish.
    Abstract: INTRODUCTION: The transmissible spongiform encephalopathies affect several species of higher animals apart from man. Amongst these, undoubtedly the best known is that affecting cattle, since the association between consumption of beef and its derivatives and the appearance of a variant of Creutzfeldt-Jakob disease in humans has been established. DEVELOPMENT: This type of pathology has been well known for many years in the ovine family, particularly in sheep and goats. In spite of the establishment of hypotheses linking the cause of the appearance of spongiform encephalopathy in cows with an interspecies jump of the disease between the ovine and bovine families, this has not yet been proved. Concomitant with the epidemic of bovine spongiform encephalopathy, other species of animals were reported to have been affected by an identical disease. These included farmed mink, deer, elks, cats and two species of exotic African ungulates (the nyala and the greater kudu). All cases were described in animals kept in captivity for human consumption. Thus it would seem that there was a common cause of the disease in all cases. CONCLUSION: In this paper we describe the most relevant aspects of the appearance of this disorder in animals, including the symptoms, epidemiology and pathology typical of each specific condition.
    Descriptors: TSE, bovine spongiform encephalopathy, BSE, NvCreutzfeldt-Jakob disease, symptoms, epidemiology, pathology, captive animals, mink, cervids, cats, nyala, kudu.

46. BSE and vCJD: causes, controls and concerns. Veterinary Record 2000 Oct 7; 147(15): 405-6 ISSN: 0042-4900 ISSN: 0042-4900
    NAL call no. 41.8 V641
    Descriptors: British Veterinary Association, BSE, issues debate, NvCreutzfeldt-Jakob Disease, current state of knowledge about BSE and CJD, human health risks.

47. BSE in Portuguese cattle to that described in British cattle. The Veterinary record: journal of the British Veterinary Association. London: The British Veterinary Association. Oct. 21, 2000. v. 147 (17) p. 486-488.
    NAL call no. 41.8 V641
    Descriptors: cattle, bovine spongiform encephalopathy, lesions, central nervous system, histopathology, Portugal, United Kingdom.

48. The BSE inquiry sets out its findings. Veterinary Record 2000 Nov 4; 147(19): 526-30 ISSN: 0042-4900
    NAL call no. 41.8 V641
    Descriptors: Phillips inquiry report, BSE, government actions, epidemic, NvCreutzfeldt-Jakob Disease, UK.

49. Burns, G.L. Medical device associated infections. ASAIO Journal. 2000. v. 46 (6) p. S1.
    Descriptors: iatrogenic transmission, medical instrumentation, infections, antibiotic resistance, immune response, bacterium adherence, prion disease, BSE, bovine spongiform encephalopathy, virus transmission, human, editorial.

50. Buschmann, A; Pfaff, E; Reifenberg, K; Muller, HM; Groschup, MH; Groschup, MH (ed.); Kretzschmar, H. Detection of cattle-derived BSE prions using transgenic mice overexpressing bovine PrPC. Prion diseases: diagnosis and pathogenesis. 2000, 75-86; 17 ref. Springer Verlag Wien; Wien; Austria. ISBN: 3-211-83530-X.
    NAL call no. QR355 A72 v. 16
    Descriptors: BSE, analytical methods, bovine spongiform encephalopathy, chimeras, detection, transgenic mouse models, infectivity, prion diseases, prion proteins, spongiform encephalopathy, transgenic.

51. Butler, D. French target reseach money at allaying BSE fears. Nature. 2000 Nov. 23; 408(6811): 392. ISSN: 0028-0836
    NAL call no. 472 N21
    Descriptors: research initiative on prions, BSE, diagnostic tests, methods of destroying meat and bone meal of infected animals, epidemiology, disease control NvCreutzfeldt-Jakob Disease, public health risks

52. Campbell, S.; Dennehy, U.; Telling, G. Analyzing the influence of PrP primary structure on prion pathogenesis in transgenic mice. Archives of virology. Supplementum. 2000. (16): 87-94. ISSN: 0939-1983.
    Abstract: Expression of prion protein (PrP) genes in transgenic (Tg) mice has been an extremely effective means of studying human and animal prion diseases. Indeed, much of what we currently understand about the molecular basis of prion pathogenesis derives from such studies. Despite these advances, the emergence of a new variant of Creutzfeldt-Jakob disease (vCJD), apparently the human manifestation of bovine spongiform encephalopathy (BSE), demonstrates that our understanding of the factors controlling prion transmission is far from complete. We review studies in Tg mice that have addressed issues of prion strains and species barriers and have provided insights into mechanisms of prion propagation. The goal of future investigation will be to determine the interplay between PrP primary structure and conformation in determining prion transmission barriers and we discuss some ongoing transgenic studies designed to address these issues.
    NAL call no. QR355 A72
    Descriptors: BSE, NvCJD, disease transmission, strains, species barriers, prion protein, transgenic mouse model, pathogenesis.

53. Carrillo, B. J; Blanco Viera, J. F; Weber, E. L. Transmissible spongiform encephalopathy (TSE). 3. Bovine spongiform encephalopathy (BSE): clinical and pathological characteristics. [Encefalopatias espongiformes transmisibles (TSE). 3. Encefalopatias espongiformes bovina (BSE): caracteristicas clinicas y patologicas.] Revista de Medicina Veterinaria (Buenos Aires). 2000. v. 81 (3) p. 225-227, 9 refs.
    NAL call no. 41.8 B86
    Descriptors: transmissible spongiform encephalopathy, bovine spongiform encephalopathy, BSE, pathology, clinical symptoms, diagnosis.

54. Casassus, B. Mad cow disease: New recruits for French prion research. Science. Dec. 1, 2000. v. 290 (5497) p. 1671 ISSN 0036-8075
    NAL call no. 470 SCI2
    Descriptors: BSE, bovine spongiform encephalopathy, prion disease, France, government, budget, Creutzfeldt Jakob disease, medical research, epidemic, Europe, cattle.

55. Chiavetta, J. A.; Sammon, A. K.; Sher, G. D.; Newman, A. M.; Ennis, M.; Gula, C. A. Donor understanding and validity of health screening questions regarding travel to countries endemic for bovine spongiform encephalopathy (BSE). Vox Sanguinis. July 2000. v. 78 (Suppl. 1) p. P344. 26th Congress of the International Society of Blood Transfusion. Vienna, Austria. July 9-14, 2000.
    NAL call no. 448.8 V94
    Descriptors: prion disease risk, blood transfusions, travel risks, BSE, bovine spongiform encephalopathy, health screening

56. Cockcroft, P.D. Clinical sign profile likelihood ratios for bovine spongiform encephalopathy suspects. Research in veterinary science. London, U.K.: W.B. Saunders Company Ltd. June 2000. v. 68 (3) p. 285-290. Includes references.
    Abstract: This study has described a method for generating the probability of a bovine spongiform encephalopathy (BSE) suspect being a true positive BSE case. A weighting equivalent to the clinical sign frequencies recorded in histopathologically confirmed BSE cases was assigned to 14 clinical observations and a clinical profile score for the case was generated by the summation of the weightings. This method was applied to 50 histopathologically confirmed (true positive cases) BSE suspects and 50 histopathologically unconfirmed (false positive cases) BSE suspects. The profile scores for the true positive BSE suspect cases were statistically significantly higher than the profile scores of the false positive BSE suspect cases (P = 0.0014) using a Mann-Whitney U non-parametric test. The mean profile score for the true positive cases was 944 and for the false positive scores was 879. Likelihood ratios for BSE suspects with different clinical profile scores were computed using different clinical profile score cut off point and ranges. A BSE suspect with profile score of 727 or above (the lowest cut off point) and 1037 or above (the highest cut off point) had likelihood ratios for being BSE of 1. 09 and 4.00 respectively. The likelihood ratios for a BSE suspect being a true positive BSE case in the profile score ranges 627-866, 867-966, 967-1036 and 1037-1067 were 0.35, 1.06, 1.30 and 4.0 respectively. Further investigations or a revisit may be justified in animals with a low probability of being BSE.
    NAL call no. 41.8 R312
    Descriptors: cattle, bovine spongiform encephalopathy, probability, clinical aspects, weighting, histopathology.

57. Cohen, F.E. Prions, peptides and protein misfolding. Molecular Medicine Today. 2000. v. 6 (7) p. 292-293
    Descriptors: prion protein, PrP, protein folding, BSE, Creutzfeldt Jakob disease, transgenic mouse models, scrapie, transmissible spongiform encephalopathies.

58. Collinge, John. Molecular biology of prion propagation. Biochemical Society Transactions. October, 2000. v. 28 (5) p. A126. 18th International Congress of Biochemistry and Molecular Biology. Birmingham, UK. July 16-20, 2000
    NAL call no. QD415 A1B58
    Descriptors: prion biology, mouse model, BSE, bovine spongiform encephalopathy, prion disease, Creutzfeldt-Jakob disease, behavioral and mental disorders, variant forms, scrapie, PrP proteins, molecular typing.

59. Conner, M.M.; McCarty, C.W.; Miller, M.W. Detection of bias in harvest-based estimates of chronic wasting disease prevalence in mule deer. J Wildl Dis. Lawrence, Kan. : Wildlife Disease Association Inc. Oct 2000. v. 36 (4) p. 691-699. ISSN: 0090-3558
    Abstract: Diseased animals may exhibit behavioral shifts that increase or decrease their probability of being randomly sampled. In harvest-based sampling approaches, animal movements, changes in habitat utilization, changes in breeding behaviors during harvest periods, or differential susceptibility to harvest via behaviors like hiding or decreased sensitivity to stimuli may result in a non-random sample that biases prevalence estimates. We present a method that can be used to determine whether bias exists in prevalence estimates from harvest samples. Using data from harvested mule deer (Odocoileus hemionus) sampled in northcentral Colorado (USA) during fall hunting seasons 1996-98 and Akaike's information criterion (AIC) model selection, we detected within-yr trends indicating potential bias in harvest-based prevalence estimates for chronic wasting disease (CWD). The proportion of CWD-positive deer harvested during fall hunting seasons. Detection of bias may provide information about temporal patterns of a disease, suggest biological hypotheses that could further understanding of a disease, or provide wildlife managers with information about when diseased animals are more or less likely to be harvested. Although AIC model selection can be useful for detecting bias in data, it has limited utility in determining underlying causes of bias. In cases where bias is detected in data using such model selection methods, then design-based methods (i.e., experimental manipulation) may be necessary to assign causality.
    NAL call no. 41.9 W64B
    Descriptors: mule deer, Odocoileus hemionus, prion diseases, chronic wasting disease, spongiform encephalopathy, statistical bias, wildlife diseases.

60. Cumings, J.; Collins, P.G.; Zettl, A. Peeling and sharpening multiwall nanotubes. Nature. Aug. 10, 2000. v. 406 (6796) p. 586 ISSN: 0028-0836
    NAL call no. 472 N21
    Descriptors: Creutzfeldt Jakob disease, epidemiology, prediction of mortality, epidemic, BSE, bovine spongiform encephalopathy, cattle, incidence, food contamination, meat, UK, human, major clinical study, adolescent, adult

61. Dandoy-Dron, Francoise; Benboudjema, Louisa; Guillo, Frederic; Jaegly, Alexandre; Jasmin, Claude; Dormont, Dominique; Tovey, Michael G.; Dron, Michel. Enhanced levels of scrapie responsive gene mRNA in BSE-infected mouse brain. Molecular Brain Research. March 10, 2000. v. 76 (1) p. 173-179
    Abstract: The expression of the mRNA of nine scrapie responsive genes was analyzed in the brains of FVB/N mice infected with bovine spongiform encephalopathy (BSE). The RNA transcripts of eight genes were overexpressed to a comparable extent in both BSE-infected and scrapie-infected mice, indicating a common series of pathogenic events in the two transmissible spongiform encephalopathies (TSEs). In contrast, the serine proteinase inhibitor spi 2, an analogue of the human alpha-1 antichymotrypsin gene, was overexpressed to a greater extent in the brains of scrapie-infected animals than in animals infected with BSE, reflecting either an agent specific or a mouse strain specific response. The levels of spi 2 mRNA were increased during the course of scrapie prior to the onset of clinical signs of the disease and the increase reached 11 to 45 fold relative to uninfected controls in terminally ill mice. Spi 2, in common with four of the other scrapie responsive genes studied, is known to be associated with pro-inflammatory processes. These observations underline the importance of cell reactivity in TSE. In addition, scrg2 mRNA the level of which is enhanced in TSE-infected mouse brain, was identified as a previously unrecognized long transcript of the murine aldolase C gene. However, the level of the principal aldolase C mRNA is unaffected in TSE. The increased representation of the longer transcript in the late stage of the disease may reflect changes in mRNA processing and/or stability in reactive astrocytes or in damaged Purkinje cells.
    Descriptors: FVB/N mouse models, BSE, bovine spongiform encephalopathy, BSE, experimental infection, scrapie, mRNA of scrapie responsive genes, brain tissue.

62. DeArmond, S.J. Cerebral amyloidosis in prion diseases. Amyloid. 2000. v. 7 (1) p. 3-6
    Descriptors: prion protein, PrP, amyloid neuropathy, amyloidosis, Creutzfeldt Jakob disease, nerve degeneration, protein conformation and structure, scrapie, BSE, Gerstmann Straussler Scheinker Syndrome, bovine spongiform encephalopathy, Alzheimer disease, protein domain, human tissue, mouse tissue

63. Debecker, D.; Roels, S.; Vanopdenbosch, E. TI: BSE-onderzoek: opsporen van PrPres door middel van de Bio-Rad platelia BSE kit. [BSE research: detection of PrPres using the Bio-Rad platelia BSE kit.] Vlaams Diergeneeskundig Tijdschrift. 2000, 69: 6, 382-384. ISSN: 0303-9021. In Dutch.
    NAL call no. 41.8 V84
    Descriptors: prion detection method, Platelia BSE, PrPres, cattle.

64. A department in transition. The Veterinary record: journal of the British Veterinary Association. London: The British Veterinary Association. April 29, 2000. v. 146 (18) p. 509. Includes references
    NAL call no. 41.8 V641
    Descriptors: government organizations, agriculture, food safety, budgets, animal welfare, animal health, veterinary services, veterinary medicine, bovine spongiform encephalopathy, scrapie, zoonoses, veterinary products, farms, diagnosis, disease control, UK.

65. De Silva, R. N. Variant Creutzfeldt-Jakob disease: an update. Hospital medicine. Feb 2000. 61(2): 82-83. ISSN: 1462-3935.
    Descriptors: Age of onset, Creutzfeldt-Jakob Syndrome, transmission, therapy, complications, bovine spongiform encephalopathy, human, phenotype.

66. Desselberger, U. Emerging and re-emerging infectious diseases. Journal of Infection. 2000. v. 40 (1) p. 3-15, 195 refs.
    Descriptors: bovine spongiform encephalopathy, Creutzfeldt-Jakob Disease, hepatitis C, hepatitis E, HIV infections, human diseases, infectious diseases, Lyme disease, viral diseases, bacterial diseases, cryptosporidiosis, reviews, HTLV infections, hepatitis F virus, hepatitis G, hepatitis G virus, GB virus, GB virus C, small round structured viruses, TT virus, human herpesvirus 8, human enterovirus 71, emerging infectious diseases, transfusion transmitted virus, Astrovirus, Bartonella, Borrelia burgdorferi, Bunyavirus, Cryptosporidium, Ebola virus, Enterovirus, Escherichia coli, Flavivirus, hepatitis C virus, hepatitis E virus, human immunodeficiency virus, human T-cell lymphotropic virus, Legionella, man, Paramyxovirus, Parvovirus B19, Vibrio cholerae, viruses, Rotavirus, human herpesvirus 6, human herpesvirus 7, Influenza virus, prions, Campylobacter, Helicobacter pylori, Ehrlichia.

67. Detwiler, L.A; Rubenstein, R. Bovine spongiform encephalopathy: An overview. ASAIO Journal. 2000. v. 46 (6) p. S73-S79
    Abstract: Bovine spongiform encephalopathy (BSE), widely known as "mad cow disease," is a chronic, degenerative disease affecting the central nervous system of cattle. Worldwide, there have been more than 180,000 cases since the disease was first diagnosed in 1986 in Great Britain. Bovine spongiform encephalopathy has had a substantial impact on the livestock industry in the United Kingdom. The disease has also been confirmed in native-born cattle in Belgium, Denmark, France, Ireland, Luxembourg, Liechtenstein, The Netherlands, Northern Ireland, Portugal, and Switzerland. However, over 95% of all BSE cases have occurred in the United Kingdom. Bovine spongiform encephalopathy is not known to exist in the United States.
    Descriptors: BSE, topical review, cattle, impacts on livestock industry, incidence, epidemiology, etiology, UK, Belgium, Denmark, France, Ireland, Luxembourg, Liechtenstein, The Netherlands, Northern Ireland, Portugal, Switzerland.

68. Dickson, D. UK ministry under fire over handling of BSE research. Nature. 2000 Oct 26; 407(6807): 932 ISSN: 0028-0836
    NAL call no. 472 N21
    Descriptors: BSE, bovine spongiform encephalopathy, research programs, UK Ministry, criticism.

69. Diringer, Heino. Bovine spongiform encephalopathy (BSE) and public health. In: Risk assessment in the food chain of children. Nestle Nutrition Workshop Series. Aggett Peter J, Kuiper Harry A. Philadelphia, PA: Lippincott Williams & Wilkins, 2000. v. 44. p. 225-233. ISBN: 0-7817-2417-1
    NAL call no. RC620 A1N47
    Descriptors: BSE, bovine spongiform encephalopathy, prion diseases, risk analysis for humans, Creutzfeldt-Jakob Syndrome.

70. Dixon, B. Hindsight blinkers Britain's mad cow disease response. Current Biology 2000 Nov 30; 10(23): R847-8 ISSN: 0960-9822
    NAL call no. QH301 C85
    Descriptors: BSE, cattle prion disease, epidemic, Phillips report of inquiry, government response, UK.

71. Dobson, R. Scientists show that vCJD can be transmitted through blood. British Medical Journal. Sep. 23, 2000. v. 321 (7263) p. 721
    NAL call no. R31 B55
    Descriptors: BSE, Nv Creutzfeldt Jakob disease, blood transfusion, bovine spongiform encephalopathy, disease transmission, sheep, United Kingdom, Red Cross, Australia, blood donor, nonhuman, animal model.

72. Doherr, M.G.; Baumgarten, L.; Heim, D. The need for an active (targeted) surveillance system for BSE and scrapie in addition to the mandatory reporting of clinical suspect cases. Proceedings of a meeting Society for Veterinary Epidemiology and Preventive Medicine. [Meeting held on March 29-31, 2000, Edinburgh] [Great Britain]: The Society, 1983-. 2000. p. 198-203. Includes references
    Descriptors: cattle, sheep, goats, bovine spongiform encephalopathy, scrapie, disease surveys Switzerland.

73. Donnelly, C.A. Likely size of the French BSE epidemic. Nature. London : Macmillan Magazines Ltd. Dec 14, 2000. v. 408 (6814) p. 787-788. ISSN: 0028-0836
    NAL call no. 472 N21
    Descriptors: bovine spongiform encephalopathy, incidence, cattle, France

74. Dreux, C.; Launay, J. M.; Laplanche, J. L. Donnees recentes sur les maladies a prions. [Recent data on prion diseases]. Bulletin et memoires de l'Academie royale de medecine de Belgique. 2000. 155(1-2): 99-108; discussion 108-111. ISSN: 0377-8231. In French.
    Abstract: The transmissible spongiform encephalopathies (TSEs) or "prions diseases", provokes some controversies about their origins and possible transmission from animals to humans. Important advances were obtained recently on the genetical TSEs with the genotypic diagnosis methods based on the discovery of numerous mutations and inclusions in the PRNP gene coding for prion protein. The possible connection between the bovine spongiform encephalopathy (BSE) and the newvariant of Creutzfeldt-Jakob disease (nv-CJD) ask numerous questions in terms of public health. Lastly, the important problem of biological diagnosis of "prions diseases" presents interesting advances that must be continued and supported by the public health authorities.
    Descriptors: TSE, origins, interspecies transmission, BSE, NvCJD, public health concerns.

75. Ducrot C.; Calavas, D.; Baron, T.; Agrech, A.E.; Coudert, M.; Savey, M. Situation epidemiologique de l'ESB en France -- point sur les cas NAIF. [Epidemiological situation [on 1 July 2000] regarding BSE in France -- comments on the BABM case [animals born after the banning of animal meals]] 7emes Rencontres autour des recherches sur les ruminants, Paris, France, 6-7 Decembre 2000. 2000, 7 (supplement), 5-8; 6 ref. Institut National de la Recherche Agronomique (INRA); Paris; France. In French.
    NAL call no. SF191.2 R46
    Descriptors: epidemiology, cattle feeding, BSE, bovine spongiform encephalopathy, cattle diseases, calves, prion diseases

76. Edskes, H.K.; Wickner, R.B. A protein required for prion generation: [URE3] induction requires the Ras-regulated Mks 1 protein. Proceedings of the National Academy of Sciences of the United States of America. June 6, 2000. v. 97 (12) p. 6625-6629 ISSN: 0027-8424
    NAL call no. 500 N21P
    Descriptors: prions, Creutzfeldt Jakob disease, Saccharomyces cerevisiae, Ure2p, cellular regulatory mechanisms, prion generation

77. The efficacy and safeness of thmus preparations are not substantiated. [Thymuspraparate: wirksamkeit und unbedenklichkeit sind nicht belegt.] Deutsche Apotheker Zeitung. March 23, 2000. v. 140 (12) p. 46-47
    NAL call no. 396.8 SU2
    Descriptors: thymus extract safety risks, BSE, bovine spongiform encephalopathy, transmissible spongiform encephalopathies, drug safety

78. Epstein, J.S. The U.S. blood supply. American Family Physician. Jan. 15, 2000. v. 61 (2) p. 549-550
    Descriptors: mass screening of blood supplies, BSE, bovine spongiform encephalopathy, prevention, disease transmission risks, Food and Drug Administration, socioeconomics, Creutzfeldt Jakob disease, US

79. Escudero Torrella, J. Cronologia de la nueva variante de la enfermedad de Creutzfeldt-Jakob. [Chronology of the new variant of Creutzfeldt-Jakob disease]. Revista de neurologia. Jul 16-31, 2000. 31(2): 141-147. ISSN: 0210-0010. In Spanish.
    Abstract: INTRODUCTION: The appearance of a new variant of Creutzfeldt-Jakob disease, and the particular conditions of this appearance, have caused a minor scientific and social upheaval in Europe. The possible relation between it and the epidemic of bovine spongiform encephalopathy in the United Kingdom led to a real scientific 'race against time' to discover the agent causing the two conditions and a possible link between them, and made the rare group of diseases known as prionic diseases fashionable. DEVELOPMENT: The aim of this article is to describe the development of events concerning the appearance of this condition, considering them objectively from the point of view of an outside observer. We discuss the different phases of the story, from the initial perplexity at the appearance of the epidemic of bovine spongiform encephalopathy, followed by uncertainty when the new variant of Creutzfeldt-Jakob disease was described and the possible relation between the two conditions. CONCLUSION: Everything concerning this process, including the different attitudes and opinions of the social, political and scientific groups involved, could form the plot of a top best selling novel of the moment.
    Descriptors: Europe, NvCJD, historical review, appearance of BSE in cattle, prion diseases, social upheaval.

80. Evatt, B. Creutzfeldt-Jakob Disease and haemophilia: Assessment of risk. Haemophilia. 2000. v. 6 (Suppl. 1) p. 94-99
    Descriptors: Creutzfeldt Jakob disease, hemophilia, BSE, bovine spongiform encephalopathy, disease transmission, heredity, infection transmission risk assessment, humans

81. Fontana, M. C.; Tamba, M.; Bardasi, L.; Bacchiocchi, F. TSE surveillance: preliminary results of control activities on ruminant feed in Emilia Romagna region of Italy. [Sorveglianza sulle TSE: risultati preliminari dell'attivita di controllo sugli alimenti destinati ai ruminanti in Emilia Romagna.] Selezione Veterinaria. 2000. (Suppl.) p. s121-s126. From: 1 deg Assemblea Annuale dei Soci ed Evento Scientifico. Salsomaggiore, Italy, 13 Novembre, 1998.
    NAL call no. 241.71 B75
    Descriptors: feed ban, animal products in feeds, contamination, control, fish meal, bovine spongiform encephalopathy, factories, bones, cattle diseases, national program for surveillance, Emilia Romagna, Italy.

82. Foster, P. R. Prions and blood products. Annals of medicine. Oct 2000. 32(7): 501-513. ISSN: 0785-3890.
    Abstract: The transmission of Creutzfeldt-Jakob disease (CJD) by human pituitary-derived growth hormone has led to concerns that blood products might also provide a route for the iatrogenic transmission of CJD. A number of actions have been implemented by regulatory authorities to address such concerns, and numerous studies have been undertaken to determine whether or not there is a risk of CJD being transmitted in this manner. To date, no excess risk has been identified, leading to a growing consensus that there is little or no risk of long established forms of CJD being transmitted to recipients of blood products. This opinion does not extend to new variant CJD (vCJD) which is found predominantly in the UK and is believed to have resulted from the transmission of bovine spongiform encephalopathy (BSE) to humans. Unlike that of CJD, the prevalence of vCJD is not known. In addition, the detection of abnormal prion protein in the tonsils of vCJD-infected individuals has led to speculation that blood infectivity may be greater than in patients with CJD. A number of precautionary measures have been taken to address the possibility that vCJD may be transmissible by blood products; however, further scientific advances are needed to enable this risk to be defined. A suitable screening test is required to identify any infected blood donors, particularly where cellular blood components are being derived from populations believed to be at risk from BSE infection. Recent experimental data suggest that process operations used in the manufacture of plasma products may be capable of removing prion agents to a significant extent. However, further work is required to confirm these observations and to determine whether or not all potential vCJD infectivity would be removed by these means.
    Descriptors: NvCJD transmission, contaminated blood and bovine based products, risks, treatment of plasma products.

83. Foucher, Pierre. Symposium scientifique international sur la securite des aliments--optimisation de la productivite et de la securite alimentaire. [France?]: Balou Communication, [2000].
    NAL call no. TP368.S96 2000
    Descriptors: food industry and trade, food quality, foodborne diseases, bovine spongiform encephalopathy, food safety.

84. Fournier, J. G. Introduction to histological localization of prion proteins. Microscopy Research and Technique. July 1, 2000. v. 50 (1) p. 1
    NAL call no. QH212 E4J69
    Descriptors: prion protein, PrP, isoprotein, histological methods, diagnosis, BSE, bovine spongiform encephalopathy, immunogold staining

85. France. Agence francaise de securitesanitaire des aliments (AFSSA). Reevaluation du dispositif francais de prevention de l'ESB: resume des travaux (saisine du 2 novembre 1999). [Reevaluation of the French position regarding the prevention of BSE: summary of studies (session of 2 November 1999).] 7emes Rencontres autour des recherches sur les ruminants, Paris, France, 6-7 Decembre-2000. 2000, 7 (supplement), 30-37. Institut National de la Recherche Agronomique (INRA); Paris; France. In French.
    NAL call no. SF191.2 R46
    Descriptors: applied research, risk assessment, bovine spongiform encephalopathy, nervous system diseases, cattle diseases, prion diseases, prevention, slaughter

86. France. Assemblee Permanente des Chambres d'Agriculture. La crise de l'ESB: situation des marches et perspectives. [The BSE crisis: market situation and prospects.] Chambres d'Agriculture. 2000, No. 894, 2-4. ISSN: 0396-7883. In French.
    NAL call no. 14 T69
    Descriptors: BSE, impacts on beef consumption, meat prices, impacts on farms, France

87. France. Assemblee Permanente des Chambres d'Agriculture. Pour repondre a la crise: les mesures nationales et europeennes. Responding to the crisis: national and European measures. Chambres d'Agriculture. 2000, No. 894, 5-7. ISSN: 0396-7883. In French.
    NAL call no. 14 T69
    Descriptors: legislation to control bovine spongiform encephalopathy, cattle diseases, prion diseases, food safety, disease control measures, France, EU.

88. France. Direction generale de l'alimentation. Bovine spongiforme [sic] encephalopathy in France: few numbers and data: appendix. [Paris?]: Republic of France, Ministry of Agriculture and Fisheries, General Directorate for Food, [2000].
    NAL call no. SF967.S63 B6842 2000
    Descriptors: Bovine spongiform encephalopathy, animal diseases, food safety, statistics, France.

89. France. Institut National de la Recherche Agronomique (INRA). 7emes Rencontres Recherches Ruminants, Paris, 6-7 decembre 2000. [7th meeting on ruminant research, Paris, France, 6-7 December 2000.] 7emes Rencontres autour des recherches sur les ruminants, Paris, France, 6-7 decembre 2000. 2000, 7 (supplement), 44 pp.; Many ref. Institut National de la Recherche Agronomique (INRA); Paris; France. In French.
    NAL call no. SF191.2 R46
    Descriptors: workshop, BSE crisis, France, prion diseases, NvCreutzfeldt-Jakob Disease, human health risks, food borne diseases.

90. Fraser, H. Phillips report and the origin of BSE. Veterinary Record. 2000 Dec 16; 147(25): 724. ISSN: 0042-4900
    NAL call no. 41.8 V641
    Descriptors: BSE, bovine spongiform encephalopathy, Nv Creutzfeldt-Jacop disease, UK government, Spongiform Encephalopathy Advisory Committee, safety of beef products, role of scientific advisory committees in policy, interdepartmental rivalry, Chief Medical Officer, Chief Veterinary Officer, compensation and care for NvCJD patients, scrapie, cattle feed bans, sheep, research initiatives, Lord Phillips report of public inquiry

91. Fraser, H.; Martin, W.B. (ed.); Aitken, I.D. Scrapie in sheep and goats, and their related diseases. Diseases of sheep. 2000, Ed.3, 207-218; 51 ref. Published by: Blackwell Science; Oxford; UK ISBN: 0-632-05139-6
    NAL call no. SF968 D57 2000
    Descriptors: sheep, scrapie, prion diseases, transmissible spongiform encephalopathies, BSE.

92. Fronte, M. Come e difficile prevedere la mucca pazza. [How difficult it is to prevent mad cow disease]. Epidemiologia e prevenzione. Sep-Oct 2000. 24(5): 201-202. ISSN: 1120-9763. In Italian.
    Descriptors: BSE, bovine spongiform encephalopathies, epidemiology, prevention, control.

93. Froslie, A; Fossum, K. Prion diseases. (Special Issue). [Prionjukdommer.] Norsk Veterinaertidsskrift. 2000. v. 112 (5) 446 pp. Special issue of 13 papers on a variety of topics including scrapie, bovine spongiform encephalopathy, and Creutzfeldt-Jakob Disease prion diseases, epidemiology of scrapie in Norway and Iceland, control programs, incidence of prion disease in Norway, BSE in Europe and Norwegian surveillance programs.
    NAL call no. 41.8 N81
    Descriptors: scrapie, prion diseases, bovine spongiform encephalopathy, BSE, Creutzfeldt-Jakob Disease, transmissible spongiform encephalopathy, reviews cattle, sheep, man, Norway.

94. Gamage, R. Prion diseases: A major challenge for future research. Ceylon Medical Journal. March 2000. v. 45 (1) p. 3-5
    Descriptors: prion diseases, research, Creutzfeldt-Jakob Disease, BSE, bovine spongiform encephalopathy, familial fatal insomnia, kuru, scrapie, transmissible spongiform encephalopathy.

95. Ganter, M.; Rehage, J.; Kaske, M. Consequences of the new Regulation on the Surveillance of Transmissible Spongiform Encephalopathies for the veterinarian. [Konsequenzen der neuen "Verordnung zur Uberwachung Transmissibler Spongiformer Enzephalopathien" fur den Tierarzt.] Praktische Tierarzt. 2000. v. 81 (1) p. 44-58, 20 refs.
    NAL call no. 41.8 P882
    Descriptors: spongiform encephalopathy, legislation, surveillance, disease control, disease prevention, bovine spongiform encephalopathy, scrapie, human diseases, zoonoses, nervous system diseases, man, sheep, cattle, Germany.

96. Garcia, F. L.; Zahn, R.; Riek, R.; Wuthrich, K. NMR structure of the bovine prion protein. Proceedings of the National Academy of Sciences of the United States of America. 2000. v. 97 (15) p. 8334-8339, 44 refs. ISSN: 0027-8424
    NAL call no. 500 N21P
    Descriptors: prion proteins, polypeptides, prion diseases, mature bovine prion protein, bPrP(23-230), sturcture, nuclear magnetic resonance, species barrier, electrostatic charges, bovine spongiform encephalopathy, cattle, man, golden hamsters.

97. Gavaghan, H. U.K. mad cow disease. Report flags hazards of risk assessment. Science. Nov 3, 2000. 290(5493): 911-913. ISSN: 0036-8075.
    NAL call no. 470 SCI2
    Descriptors: Animal, feed, cattle, Creutzfeldt-Jakob Syndrome, transmission, prevention and control, epidemiology, Great Britain, human, risk assessment.

98. Germain, M.; Decary, F.; Chiavetta, J.; Goldman, M. Variant Creutzfeldt-Jakob Disease and the Quebec blood supply. Canadian Medical Association Journal. Aug. 22, 2000. v. 163 (4) p. 412-413
    NAL call no. R11 C3
    Descriptors: NvCreutzfeldt-Jakob disease, BSE, bovine spongiform encephalopathy, blood donor, prion diseases, epidemic, United Kingdom, virus transmission, blood transfusion, blood banks, Canada

99. Gerstoft, J. Human smitte med bovin spongiform encefalopati prioner. [Transmission of bovine spongiform encephalopathy prions to human] Ugeskrift for Laeger 2000 Aug 21; 162(34): 4515-7 ISSN: 0041-5782. In Danish.
    Descriptors: BSE, transmissibility, NvCruetzfeldt-Jakob-Disease, contaminated bovine-based food products, beef, humans, prion diseases.

100. Ghani, A.C.; Ferguson, N.M.; Donnelly, C.A.; Anderson, R.M. Predicted VCJD mortality in Great Britain. Nature. London: Macmillan Magazines Ltd. Aug. 10, 2000. v. 406 (6796) p. 583-584. Includes references.
     NAL call no. 472 N21
     Descriptors: Creutzfeldt-Jakob Disease, mortality, prediction, bovine spongiform encephalopathy, Great Britain.

101. Ghani, A. C.; Ferguson, N. M.; Donnelly, C. A.; Anderson, R. M. Scrapie in Britain during the BSE years. Nature (London). 2000. v. 406 (6796) p. 584-585, 10 refs. ISSN: 0028-0836
     NAL call no. 472 N21
     Descriptors: bovine spongiform encephalopathy, BSE, scrapie, incidence, epidemiology, farm survey, sheep, cattle, UK.

102. Gibbens, J. C.; Wilesmith, J. W. Veterinary surveillance and the role of epidemiology. State Veterinary Journal. 2000. v. 10 (1) p. 9-11
     NAL call no. SF601 S8
     Descriptors: epidemiology, disease surveys, animal diseases, veterinary services, BSE, bovine spongiform encephalopathy, diagnosis

103. Gilch, S.; Spielhaupter, C.; Schatzl, H.M. Shortest known prion protein allele in highly BSE-susceptible lemurs. Biological Chemistry. 2000. v. 381 (5-6) p. 521-523
     NAL call no. QP501 B56
     Descriptors: prion protein, PrP, infectibility of lemurs, allele, disease susceptibility.

104. Glatzel, Markus; Aguzzi, Adriano. Peripheral pathogenesis of prion diseases. Microbes and Infection. May, 2000. v. 2 (6) p. 613-619
     Abstract: Prions are infectious pathogens that cause a group of neurodegenerative diseases characterized by spongiform degeneration of the central nervous system. Prions appear to lack informational nucleic acid. The most notable prion diseases include bovine spongiform encephalopathy, scrapie in sheep and Creutzfeldt-Jakob Disease of humans. Transmission is thought to be achieved through conversion of a normal host protein into a pathological isoform. Although the main pathological changes occur in the brain, the infectious agent accumulates early in lymphoid tissue. The development of clinical disease depends on an intact immune system including mature B-cells. The authors review the current understanding of the routes of neuroinvasion used by the infectious agent to gain access to the central nervous system upon entry into extracerebral sites.
     NAL call no. QR180 M53
     Descriptors: BSE, TSE, bovine spongiform encephalopathy, cattle, scrapie, sheep, Bâcells, Creutzfeldt-Jakob Disease of humans, paths of invasion, immune system.

105. Glatzel, M; Klein, MA; Brandner, S; Aguzzi, A; Groschup, MH (ed.); Kretzschmar, H. Prions: from neurografts to neuroinvasion. Prion diseases: diagnosis and pathogenesis. 2000, 3-12; 39 ref. Springer Verlag Wien; Wien; Austria. ISBN: 3-211-83530-X.
     NAL call no. QR355 A72 v. 16
     Descriptors: BSE, bovine spongiform encephalopathy, central nervous system, neurons, pathogenesis, peripheral nerves, prion diseases and proteins, reticuloendothelial system, scrapie, spongiform encephalopathy.

106. Gonzalo Pascual, I.; Cuadrado Corrales, N. Neuropatologia de la nueva variante de la enfermedad de Creutzfeldt-Jakob. [Neuropathology of the new variant of Creutzfeldt-Jakob disease]. Revista de neurologia Jul 16-31, 2000. 31(2): 160-261. ISSN: 0210-0010. In Spanish.
     Abstract: Identification in the United Kingdom of cases of Creutzfeldt-Jakob disease with clinical, genetic, neuropathological and phenotype characteristics different from those previously reported, together with its relation to bovine spongiform encephalopathy led to the establishment of a new condition known as a new variant of Creutzfeldt-Jakob disease. In this article we describe the neuropathology and immunohistochemistry of the prion protein involved.
     Descriptors: BSE, NvCJD, neuropathology, immunohistochemistry, prion protein.

107. Grassi, J.; Creminon, C.; Frobert, Y.; Fretier, P.; Turbica, I.; Rezaei, H.; Hunsmann, G.; Comoy, E.; Deslys, J. P. Specific determination of the proteinase K-resistant form of the prion protein using two-site immunometric assays. Application to the post-mortem diagnosis of BSE. Archives of virology. Supplementum. 2000. (16): 197-205. ISSN: 0939-1983.
     Abstract: The aim of this work was to establish an immunological test suitable for specifically detecting PrPres in tissues from animals or humans developing TSEs. We chose to use as detection method a conventional two-site immunometric assay (sandwich immunoassay) because over the last 20 years this technique has clearly been shown to be more sensitive and specific than other tests. We have established numerous two-site immunometric assays based on the use of monoclonal antibodies and suitable for measurement of PrPsen in various mammalian species (human, bovine, ovine, mouse and hamster). A detection limit below 100 pg/ml was estimated from standard curves established using ovine recombinant PrP. PrPres was selectively detected by processing samples (currently brain homogenates) to enable specific purification and concentration of PrPres, which was finally solubilized by a strong denaturing treatment. This sample-processing procedure can be achieved within 30 minutes. The capacity of this test to detect bovine PrPres was estimated in the framework of an evaluation study organized by the Directorate-General XXIV of the European Commission during May 1999. On this occasion, a blind test on 1400 brain stem samples taken from either healthy (1000) or BSE-infected (300) cows demonstrated 100% sensitivity and specificity. In addition, dilution experiments showed that the test can significantly detect PrPres in homogenates diluted 1/300 and was at least as sensitive as a conventional bioassay performed on mice.
     NAL call no. QR355 A72
     Descriptors: prion protein, PrPes, immunologically based diagnostic tests, two-site immunometric assay, monoclonal antibodies, cattle assay.

108. Gravenor, M.B.; Cox, D.R.; Hoinville, L.J.; Hoek, A.; McLean, A.R. Scrapie in Britain during the BSE years. Nature. London: Macmillan Magazines Ltd. Aug. 10, 2000. v. 406 (6796) p. 584-585. Includes references
     NAL call no. 472 N21
     Descriptors: scrapie, infections, sheep, bovine spongiform encephalopathy, disease incidence, Great Britain.

109. Great Britain. Her Majesty's Stationery Office. The BSE inquiry: the report: the inquiry into BSE and variant CJD in the United Kingdom. [London : Stationery Office], c2000. Includes bibliographical references.
     Abstract: Describes the emergence and identification of bovine spongiform encephalopathy and variant Creutzfeldt-Jakob disease in the United Kingdom.
     NAL call no. SF967.S63B746 2000
     Descriptors: Bovine spongiform encephalopathy, Creutzfeldt-Jakob disease, Great Britain.

110. Great Britain. Ministry of Agriculture, Fisheries and Food. Bovine spongiform encephalopathy in Great Britain: a progress report. [Tolworth : Ministry of Agriculture, Fisheries and Food, England]
     NAL call no. SF967.S63-B683
     Descriptors: Bovine spongiform encephalopathy, periodicals, Cattle virus diseases, Great Britain, Creutzfeldt-Jakob disease, animal-diseases, law, regulations, United Kingdom

111. Great Britain. Ministry of Agriculture, Fisheries and Food. BSE enforcement bulletin. Tolworth: Ministry of Agriculture, Fisheries and Food, 1966-
     NAL call no. SF967.S63 B743
     Descriptors: Bovine spongiform encephalopathy, periodicals, cattle virus diseases, Creutzfeldt-Jakob Disease, animal diseases, law and regulations, food safety research, Great Britain, United Kingdom.

112. Great Britain. Minisry of Agriculture, Fisheries and Food. BSE, measures taken by the UK. [Tolworth : Ministry of Agriculture, Fisheries and Food, England]
     NAL call no. SF967.S63-B744
     Descriptors: Bovine spongiform encephalopathy, periodicals, Cattle virus diseases, Great Britain, Creutzfeldt-Jakob disease, animal-diseases, law, regulations, United-Kingdom.

113. Groschup, M. H.; Kuczius, T.; Junghans, F.; Sweeney, T.; Bodemer, W.; Buschmann, A. Characterization of BSE and scrapie strains/isolates. Archives of virology. Supplementum. 2000. (16): 217-226. ISSN: 0939-1983.
     Abstract: Following the BSE epidemic in cattle and the emergence of a variant form of Creutzfeldt-Jakob disease in humans, the question was raised whether BSE has been transmitted to small ruminants by the inadvertent feeding of infectious meat and bone meal. Such infections could easily be concealed in countries where scrapie is endemic. To address this issue by immuno-chemically analyzing the PrP(Sc) fragments, we have developed two lines of research. Firstly we have focused on the development of criteria for the differential characterization of experimental BSE and scrapie strains/isolates in rodents. To date, three criteria have been identified: quantification of the relative banding intensities of PrP(Sc) glycotypes using a photoimaging technique; the non-uniform kinetic of proteinase K degradation of PrP(Sc); and differences in the molecular masses of their non-glycosylated PrP(Sc) fragments after PK cleavage in immunoblot. The second line of research focused on the implementation of the criteria described above to representative samples from scrapie diseased Irish sheep. Using these three criteria, no evidence was found for the presence of a BSE infection in these animals. However, the final conclusion must take into account the results of mouse incubation time and mouse lesion profile data which are currently being generated.
     NAL call no. QR355 A72
     Descriptors: BSE, NvCreutzfeldt-Jakob Disease, prion protein, PrPSc fragment analysis, comparative study, strain isolates, scrapie infected sheep.

114. Groschup, M.H.; Pfaff, E.; Reifenberg, K.; Buschmann, A. Conventional and transgenic mouse models for the detection of ruminant-derived BSE and scrapie infectivity. Infection. November, 2000; 28 (Supplement 1): 24. ISSN: 0300-8126. 1st Congress on Viral Diseases ConVir 2000, Munich, Germany, October 10-12, 2000.
     Descriptors: experimental animal models, mouse models, transgenic animal, BSE, scrapie

115. Groschup, MH; Kretzschmar, H; Groschup, MH (ed.); Kretzschmar, H. Prion diseases: diagnosis and pathogenesis. 2000, 290 pp. Springer-Verlag Wien; Wien; Austria. ISBN: 3-211-83530-X. 27 chapter book on a variety of topics.
     NAL call no. QR355 A72 v. 16
     Descriptors: analytical methods, prion proteins, bovine spongiform encephalopathy, NvCreutzfeldt Jakob disease, detection, diagnostic, techniques, differential diagnosis, disease models and surveys, epidemiology, epitopes, histochemistry, histopathology, disease incidence, infectivity, localization, mass spectrometry, monoclonal antibodies, neurofilaments, neurons, pathogenesis, proteinases, reticuloendothelial system, scrapie, spleen, transmissible spongiform encephalopathy, transgenic animals, transgenics, diagnostic techniques.

116. Hagen, G.; Thorud, K. E.; Hoel, K.; Stovring, M. Surveillance and control programmes for transmissible spongiform encephalopathies. [Skrapesjuke og andre over-forbare spongiforme encefalopatier Forvaltningsmessige forhold i Norge.] Norsk Veterinaertidsskrift. 2000. v. 112 (5) p. 381-385, 18 refs. Special issue of 13 papers on a variety of topics including scrapie, bovine spongiform encephalopathy, and Creutzfeldt-Jakob Disease prion diseases, epidemiology of scrapie in Norway and Iceland, control programs, incidence of prion disease in Norway, BSE in Europe and Norwegian surveillance programs.
     NAL call no. 41.8 N81
     Descriptors: scrapie, disease control, surveillance programs, prion diseases, reviews, sheep, Norway.

117. Hagenaars, T.J.; Ferguson, N.M.; Donnelly, C.A.; Ghani, A.C.; Anderson, R.M. Feed-borne transmission and case clustering of BSE. Proceedings. Biological sciences. London, U.K.: The Royal Society. Feb.7, 2000. v. 267 (1440) p. 205-215. Includes references
     Abstract: An unresolved issue in the epidemiology of bovine spongiform encephalopathy (BSE) in the UK is what precisely determines the degree to which cases of disease in cattle are clustered within herds throughout the course of the epidemic. This paper presents an analysis of feed-borne transmission at the herd level and tests various models of case-clustering mechanisms, associated with heterogeneity in exposure to infectious feed, against observed epidemic pattern. We use an age-structured metapopulation framework in which the recycling of animal tissue between herds via feed producers is explicitly described. We explore two alternative assumptions for the scaling with herd size of the within-herd risk of exposure of an animal to infectious material. We find that whereas exposure heterogeneity caused by variation in feed and offal processing methods and by variation in per-animal feed uptake can explain the pattern of case clustering seen in the BSE epidemic, exposure heterogeneity due to the aggregation of infectivity within feed cannot.
     NAL call no. 501 L84B
     Descriptors: bovine spongiform encephalopathy, infectivity, epidemiology, mathematical models.

118. Haltia, Matti. Human prion diseases Annals of Medicine. October, 2000. v. 32 (7) p. 493-500.
     Abstract: The term 'prion diseases' refers to a group of neurodegenerative disorders thought to be caused by prions. Prions are pathogenic agents with novel modes of replication and transmission. Prion diseases are characterized by long incubation periods ranging from months to years. Once clinical symptoms have appeared, they are invariably fatal. They are also called transmissible spongiform encephalopathies (TSE), on account of the predominant neuropathological change observed in the central nervous system. The three most important members of this group are Creutzfeldt-Jakob Disease (CJD) of man displaying sporadic, inherited and infectious forms, bovine spongiform encephalopathy (BSE, 'mad cow disease') of cattle, and scrapie of sheep. Despite their rarity, human prion diseases have recently been in the news because of the likely connection between a new variant of human CJD (vCJD) and BSE and the possibility of contamination of human blood and blood products by the vCJD agent. This short review discusses the basic biological properties of prions, followed by a presentation of the clinical and pathological features of the most important human prion diseases.
     Descriptors: prions diseases, human disease, transmission, scrapie, bovine spongiform encephalopathy, Creutzfeldt-Jakob Disease.

119. Haouet, M. N.; Rea, S.; Antonio, D. E.; Altissimi, M. S. Diagnostic approaches for the identification of animal meals. [Approcci diagnostici nell'identificazione delle farine di carne.] Selezione Veterinaria. 2000. (Suppl.) p. s375-s381. From: 1 deg Assemblea Annuale dei Soci ed Evento Scientifico. Salsomaggiore, Italy, 13 Novembre, 1998.
     NAL call no. 241.71 B75
     Descriptors: feeds, analytical methods, diagnosis, bovine spongiform encephalopathy, contamination of animal based feeds, bones, microscopy, electron microscopy, polymerase chain reaction.

120. Harash, Narang. BSE/CJD: chemical or biological? Chemistry & Industry. 2000. (No. 19) p. 630.
     NAL call no. 382 M31C
     Descriptors: prion diseases, bovine spongiform encephalopathy, Creutzfeldt-Jakob Disease, etiology, prions

121. Hardt, M.; Baron, T.; Groschup, M. H. A comparative study of immunohistochemical methods for detecting abnormal prion protein with monoclonal and polyclonal antibodies. Journal of Comparative Pathology. 2000. v. 122 (1) p. 43-53, 40 refs. ISSN: 0021-9975.
     Abstract: Transmissible spongiform encephalopathies are associated with the accumulation of abnormal prion protein (PrP(Sc)) in the central nervous system which can be detected immunohistochemically. Using a monoclonal antibody (L42) to an epitope on the first alpha-helix of ruminant PrP, we compared previously reported immunohistochemical antigen unmasking and "visualization" systems. In addition, a variety of polyclonal and monoclonal antibodies to other epitopes on ruminant PrP were assessed. Antigen unmasking by hydrated autoclaving and proteinase K treatments, and antigen detection with L42 and an avidin-biotin complex system, enabled intra- and extra-neuronal PrP(Sc)to be demonstrated in scrapie-affected sheep carrying three different PrP alleles, as well as in cases of bovine spongiform encephalopathy.
     NAL call no. 41.8 J82
     Descriptors: immunohistochemistry, monoclonal and polyclonal antibodies, prion proteins, alleles, bovine spongiform encephalopathy, BSE, scrapie, prion diseases, epitopes, extra-neuronal PrPSc, ruminant prion protein, 3 different PrP alleles, sheep, cattle.

122. Harris, D. A. Prion diseases. Nutrition. Jul-Aug 2000. 16(7-8): 554-556. ISSN: 0899-9007.
     NAL call no. QP141.A1N866
     Descriptors: Cattle, Creutzfeldt-Jakob Syndrome, bovine spongiform encephalopathy, human, prion diseases, transmission, etiology, epidemiology, prions, sheep.

123. Heim, D.; Wilesmith, J.W. Surveillance of BSE. Archives of virology. Supplementum. 2000. (16): 127-133. ISSN: 0939-1983.
     Abstract: The current method used to identify suspect BSE cases is based on reporting cattle displaying clinical signs compatible with BSE. The reporting of such cases is dependent on the ability of farmers and veterinarians to recognise the disease symptoms and on the willingness to report such cases. Furthermore, it depends on the stage of the disease, because early clinical signs of BSE are not always typical. Histology and immunohistochemistry are established and reliable to confirm BSE in cattle, but the procedure is cumbersome, time consuming and therefore not suited for mass testing of animals. A targeted surveillance system using the Prionics-Western-Blot Test was initiated in Switzerland in 1999. Prionics-positive results are confirmed by histology or immunohistochemistry by the BSE-reference laboratory. This surveillance scheme has confirmed fallen stock and cows subjected to emergency slaughter as the major risk groups. Currently all cattle from these two categories are tested. As a further measure a random sample of cows from regular slaughtering is tested. This enables to determine the BSE status independent of the inaccuracies of a clinical case reporting system. This approach may be helpful to reliably assess the BSE situation in countries with low incidence in order to verify their BSE status and in countries which want to prove their BSE-free status.
     NAL call no. QR355 A72
     Descriptors: cattle, Prionics Western Blot diagnostic test, surveillance, downer stock, testing at slaughter.

124. Herrmann, L.M.; Baszler, T.V.; Knowles, D.P. PrPc mRNA, but not PrPSc is found in the salivary glands of scrapie-infected sheep. Biochim Biophys Acta. Amsterdam : Elsevier Science B.V. June 15, 2000. v. 1479 (1/2) p. 147-154. ISSN: 0006-3002
     Abstract: Transmission studies in transmissible spongiform encephalopathies (TSEs) have become increasingly important due to the possible transmission of bovine spongiform encephalopathy to humans resulting in new variant Creutzfeldt-Jacob disease. The horizontal transmission of scrapie, a TSE of sheep, is poorly understood. Possible sources of horizontal transmission are the submandibular and parotid salivary glands. TSEs like natural sheep scrapie are characterized by the conversion of a normal protease sensitive prion protein, PrPc, to an abnormal protease resistant prion protein, PrPSc. Since the presence of PrPSc is an indicator of disease, the salivary glands of scrapie-infected sheep were examined for the presence of PrPSc. Although PrPc mRNA was detected in the salivary glands, PrPSc was not found in the salivary glands of scrapie-infected sheep. These data suggest that the salivary glands are unlikely sources of horizontal transmission of natural sheep scrapie.
     NAL call no. 381 B522
     Descriptors: sheep, scrapie, prion proteins, messenger RNA, transmissible spongiform encephalopathies, BSE, NvCreutzfeldt-Jakob Disease, salivary glands examination.

125. Herrmann, R.; Krischik-Bautz, S.; Thompson, S.R. BSE and generic promotion of beef: an analysis for 'quality from Bavaria'. Agrarokonomische Diskussionsbeitrage Universitat Giessen. 2000, No. 61, 18 pp.; 61 ref. Published by Institut fur Agrarpolitik und Marktforschung,Universitat Giessen; Giessen; Germany.
     Descriptors: beef, 'Quality from Bavaria' advertising program, cost-benefit analysis, demand, bovine spongiform encephalopathy, food safety, consumer concerns, study results, EU.

126. Herve, P. Securite transfusionnelle: risques emergents ou hypothetiques. [Transfusion safety: emergent or hypothetical risks]. Transfusion clinique et biologique journal de la Societe francaise de transfusion sanguine. Feb 2000. 7(1): 30-38. ISSN: 1246-7820. In French.
     Abstract: Three categories of emerging risks are studied: 1) A new variant of Creutzfeld-Jakob disease, different from its sporadic form; limited to the British isles (48 of 51 cases), it affects younger patients, and has a higher duration with a predominance of psychiatric symptoms. Environmental risk factors include a previous stay in the British isles and oral transmission via contaminated food. No link has been made evident between blood component (BC) transfusion and occurrence of the disease. A potential risk exists if its agent is found in blood and peripheral lymphoid tissues and if buffy coat from infected animals has been inoculated intracerebrally. Since 1993, prevention measures have been taken: exclusion of donors with a potential risk as well as transfused donors, systematic leukocyte reduction and implementation of disease surveillance. Excluding donors after a several month-stay in the British Isles is being discussed. 2) Novel hepatitis viruses. Hepatitis G virus (HGV) has been detected in 2-4% of blood donors. Ten percent of patients with chronic non-A-E hepatitis are HGV RNA positive. The incidence of HGV infection is higher than expected from PCR studies. HGV has a high prevalence in the world. Novel DNA non-enveloped virus (TTV) has a normal distribution. Its prevalence varies from 2 to 80%, depending on the country. Although it has not been shown to be aggressive for the liver, prolonged follow-up is required. 3) Human herpes virus 8 (HHV8) is associated with Kaposi's sarcoma in 80% of cases. Its prevalence (0-20%) varies depending on the country. Kaposi's sarcoma has never been reported after BC transfusion. PCR-based viral DNA searches have yielded negative results in 19 poly-transfused subjects. Continuous monitoring is required for recipients at risk (e.g., immunosuppressed). In response to a possible health risk, emerging risks govern the "Precaution Principle", so difficult to implement.
     Descriptors: NvCJD, possible blood transfusion transmission, risk control, emerging risks.

127. Hill, A.F.; Joiner, S.; Linehan, J.; Desbruslais, M.; Lantos, P.L.; Collinge, J. Species-barrier-independent prion replication in apparently resistant species. Proceedings of the National Academy of Sciences of the United States of America. Aug. 29, 2000. v. 97 (18) p. 10248-10253 ISSN: 0027-8424
     Abstract: Transmission of prions between mammalian species is thought to be limited by a "species barrier," which depends on differences in the primary structure of prion proteins in the infecting inoculum and the host. Here we demonstrate that a strain of hamster prions thought to be nonpathogenic for conventional mice leads to prion replication to high levels in such mice but without causing clinical disease. Prions pathogenic in both mice and hamsters are produced. These results demonstrate the existence of subclinical forms of prion infection with important public health implications, both with respect to iatrogenic transmission from apparently healthy humans and dietary exposure to cattle and other species exposed to bovine spongiform encephalopathy prions. Current definitions of the species barrier, which have been based on clinical end-points, need to be fundamentally reassessed.
     NAL call no. 500 N21
     Descriptors: prion diseases, BSE, transmissible spongiform encephalopathies, hamster, subclinical forms of prion infection, mice, public health risks, species barriers, kuru, Creutzfeldt Jakob disease

128. Hillier, C. E. M.; Salmon, R.L. Is there evidence for exogenous risk factors in the aetiology and spread of Creutzfeldt-Jakob Disease? QJM. Sep. 2000. v. 93 (9) p. 617-631
     Descriptors: BSE, bovine spongiform encephalopathies, Creutzfeldt-Jakob Disease, etiology, epidemiological factors, risk factors, public health.

129. Hilton, D.A. vCJD - Predicting the future? Neuropathology and Applied Neurobiology. 2000. v. 26 (5) p. 405-407
     Abstract: The recent emergence of variant Creutzfeldt-Jakob disease (vCJD) in the UK, and demonstration that vCJD is caused by the same prion strain that causes bovine spongiform encephalopathy, have led to concerns about the possibility of a human epidemic. Although only 79 cases of vCJD have occurred to date, it is likely that hundreds of thousands of infected cattle entered the human food chain in the late 1980s and early 1990s, and the average incubation period of vCJD is unknown. Mathematical models have not yet been able to give useful predictions of future numbers of cases, and in the absence of a blood test for vCJD, current attempts to reduce uncertainties about future numbers of cases are based on the accumulation of PrPSc in lymphoreticular tissues. Extensive lymphoreticular PrPSc accumulation has been seen in all cases of symptomatic vCJD so far examined, and in one case 8 months prior to the onset of symptoms. Animal models of prion disease suggest that lymphoreticular involvement occurs early in the incubation period and reliably predicts future neurological disease. Based on these data, large scale anonymous studies looking for PrP accumulation in surgically removed tonsillectomy and appendicectomy specimens are underway. Examination of the first 3000 specimens has not revealed any positive samples, but at the moment the significance of negative findings is uncertain. It is anticipated that by the time these studies are complete more data will be available on how early PrP can be demonstrated in lymphoreticular tissue in vCJD, which together with the results from examination of further samples, will allow some comment as to the likelihood of a large human vCJD epidemic.
     Descriptors: NvCreutzfeldt-Jakob Disease, BSE, bovine spongiform encephalopathy, transmissible spongiform encephalopathies, incidence, lymphoreticular involvement, disease transmission levels, PrP(Sc) accumulation testing.

130. Hinton, M H. Infections and intoxications associated with animal feed and forage which may present a hazard to human health. Veterinary Journal. March 2000. v. 159 (2) p. 124-138
     Abstract: Animal feed or forage may be the source of a limited number of infections for farm animals that could lead to human illness. Likely organisms include Salmonella enterica, Toxoplasma gondii, Trichinella spiralis and possibly the agent of bovine spongiform encephalopathy. The risk to human health from other infectious agents which may contaminate either feed or forage appear to be either negligible, e.g. Bacillus anthracis and Mycobacterium bovis, or non-existent, e.g. Clostridium botulinum toxin and Listeria monocytogenes. Mycotoxins present in animal feed can result in foods of animal origin also containing them. This risk is well recognized but has yet to be quantified accurately and in some instances the risk may be of theoretical rather than practical importance. Pesticides, agricultural and industrial chemicals, heavy metals and radionuclides may pollute animal feed and forages. The methods available for controlling pollution from these sources are well understood from a technical point of view although the effective implementation of controls can be difficult.
     NAL call no. SF601 V484
     Descriptors: feeds, forages, risks of contamination, humans, Salmonella, Toxoplasma gondii, Trichinells, BSE, bovine spongiform encephalopathy, mycotoxins, chemicals, heavy metals, readionuclides.

131. Hofmann, K. Prophylaxis of BSE by controlling meat and bone meals: the ELISA heating test and the criterias for assessment. [BSE-Prophylaxe durch Tiermehlkontrolle: Der ELISA-Erhitzungstest und seine Bewertungskriterien.] Fleischwirtschaft. 2000. v. 80 (4) p. 140-143.
     Abstract: The enables the monitoring of the effectiveness of heating in the meat meal plants and control of the "material flow". Its application in the EU would exclude inadequately heated meat meals from the feed and finally will help to eliminate the reasons for BSE. Two inter-laboratory trials, one in Germany and one elsewhere in the EU, have confirmed the practicability and reliability of the ELISA-test. The criteria for the assessment of proper heated meat meals (R<3, 1, PK<20, 4) have been established with respect to the legal regulations. A general lowering of these limitation values is neither necessary nor justified at the present situation of regulation.
     Includes 15 references.
     NAL call no. 280.38 F62
     Descriptors: ELISA meat meal heating test, legal regulations, meat and bone meals, control of processing plants, monitoring, bovine spongiform encephalopathy, animal based feeds, cattle, Germany.

132. Holloway, C. The BSE crisis in the EU and its impact on medicinal products. Pharmaceutical Technology Europe. 2000. v. 12 (5) p. 4+6. Editorial
     Descriptors: bovine-based pharmaceutical products, drug development, BSE, bovine spongiform encephalopathy, drug manufacture, disease transmission risks, good manufacturing practice, contamination and infection control, drug control, drug safety

133. Hope, James. Hunting for prions in Cyprus: Important needles in the TSE haystack. Veterinary Journal. May, 2000. v. 159 (3) p. 213-214
     NAL call no. SF601 V484
     Descriptors: transmissable spongiform encephalopathies, prions, diagnostic methods, cattle, sheep, scrapie, bovine spongiform encephalophies, Cyprus

134. Hope, James. Prions and neurodegenerative diseases. Current Opinion in Genetics & Development. October, 2000. v. 10 (5) p. 568-574.
     NAL call no. QH426 C88
     Descriptors: BSE, prion diseases, cattle, humans, transgenics, sheep, scrapie, Creutzfeldt-Jakob Disease, bovine spongiform encephalopathy.

135. Houston, F.; Foster, J. D.; Chong, Angela; Hunter, N.; Bostock, C. J. Transmission of BSE by blood transfusion in sheep. Lancet (North American Edition). Sep. 16, 2000. v. 356 (9234) p. 999-1000.
     Abstract: Authors research demonstrates it is possible to transmit bovine spongiform encephalopathy (BSE) to a sheep by transfusion with whole blood taken from another sheep during the symptom-free phase of an experimental BSE infection. BSE and variant Creutzfeldt-Jakob Disease (vCJD) in human beings are caused by the same infectious agent, and the sheep-BSE experimental model has a similar pathogenesis to that of human vCJD. Although UK blood transfusions are leucodepleted-a possible protective measure against any risk from blood transmission-this report suggests that blood donated by symptom-free vCJD-infected human beings may represent a risk of spread of vCJD infection among the human population of the UK.
     NAL call no. 448.8 L22
     Descriptors: sheep model, disease transmission by blood transfusion, BSE, bovine spongiform encephalopathies, Creutzfeldt-Jakob Disease, prion diseases, risk factors, human blood supply.

136. Hueston, William D.; Voss, James L.; Bartz, Jason; Belay, Ermias D.; Detwiler, Linda A.; Miller, Janice M.; Olander, Doris; Potter, Morris E.; Rubenstein, Richard; Schonberger, Lawrence B.; Vicari, Andrea; Williams, Elizabeth S. Transmissible spongiform encephalopathies in the United States. Council for Agricultural Science and Technology Task Force Report. October, 2000; (136): i-viii; 1-36. ISSN: 1057-7017
     NAL call no. S22.C6
     Descriptors: transmissible spongiform encephalopathies, BSE, scrapie, chronic wasting disease, deer, cattle, sheep, other mammals.

137. International Office of Epizootics. White spot disease in Panama; Bovine spongiform encephalopathy in Denmark; Foot and mouth disease in Taipei China, in goats; West Nile fever in the United States of America. Disease Information - Office International des Epizooties. 2000. v. 13 (8) p.27-31. Paris, France: Office International des Epizooties.
     NAL call no. SF781 D57
     Descriptors: emerging zoonotic diseases, bovine spongiform encephalopathy, BSE, foot and mouth disease, FMD, wild birds vectors, West Nile virus, goats, cattle, Penaeus, Panama, USA, Taiwan, Denmark.

138. Ironside, J.W. Pathology of variant Creutzfeldt-Jakob disease. Archives of virology. Supplementum. 2000. (16): 143-151. ISSN: 0939-1983.
     Abstract: Variant Creutzfeldt-Jakob disease (vCJD) is a novel prion disease in man which was first described in 1996 in the UK. There is substantial evidence to indicate that vCJD represents the effects of the bovine spongiform encephalopathy (BSE) agent in man. The neuropathology of vCJD is characterised by the florid plaque, composed of a central amyloid core with a fibrillary periphery, surrounded by a rim of spongiform change in an intact neuropil. Unique patterns of PrP accumulation in vCJD are revealed by immunocytochemistry in the cerebral and cerebellar cortices, the basal ganglia, thalamus and brainstem. The neuropathology of the thalamus and midbrain is also characterised by severe neuronal loss and gliosis. vCJD is distinct from other human prion diseases in that disease-associated PrP accumulates within follicular dendritic cells in lymphoid tissue, and consistently in peripheral sensory ganglia. All vCJD patients so far have been methionine homozygotes at codon 129 in the PrP gene. There is no evidence to indicate that cases of BSE infection have occurred in individuals in the UK who are MV or VV at codon 129 in the PrP gene. It is conceivable that BSE incubation periods in these groups may be longer than in methionine homozygotes, hence the precise numbers of future cases of vCJD are difficult to estimate at present.
     NAL call no. QR355 A72
     Descriptors: NvCreutzfeldt-Jakob Disease, BSE, prion protein accumulation pattern, estimation of future infections.

139. Ironside, J.W. Update on variant Creutzfeldt-Jakob Disease. Amyloid. 2000. v. 7 (2) p. 141-144. Editorial.
     Descriptors: NvCreutzfeldt Jakob disease, etiology, clinical features, prion diseases, BSE, bovine spongiform encephalopathy, pathogenesis, humans

140. Jackson, Graham S.; Clarke, Anthony R. Mammalian prion proteins. Current Opinion in Structural Biology. Feb., 2000. v. 10 (1) p. 69-74
     Abstract: The past two years have seen the extension of our knowledge on the cellular prion protein structure with new NMR data on both the hamster and human proteins. In addition, the folding dynamics of two cellular prion proteins have been elucidated. There are now several examples of recombinant prion proteins that are able to adopt different conformations in solution and recent work on the molecular basis of prion strains has done much to consolidate the protein-only hypothesis. Important advances in relating disease to structure have also been made through the identification of the minimal prion protein fragment that is capable of conferring susceptibility to and propagation of the scrapie agent.
     NAL call no. QH506 C86
     Descriptors: prion diseases, Creutzfeldt-Jakob Disease, bovine spongiform encephalopathy, scrapie, description, PrP.

141. Jacob, Merle; Hellstrom, Tomas. Policy understanding of science, public trust and the BSE-CJD crisis. Journal of Hazardous Materials. November 3, 2000. v. 78 (1-3) p. 303-317.
     Abstract: Discussion of how institutional factors can produce a Bovine Spongiform Encephalopathy (BSE)-Creutzfeldt-Jakob Disease (CJD) crisis in Britain are used as a case example. Risk parameters include the way policymakers understand science, and the role of precaution in issues of high uncertainty. It is pointed out that the failure to fully appreciate the complexity of the BSE-CJD situation rested on institutional situations that predisposed decision makers, at the national policy levels, to adopt a counter productive approach in handling situations of high scientific uncertainty. The article describes how these factors played out in the BSE-CJD crisis in Britian.
     NAL call no. T55.3 H3J6
     Descriptors: bovine spongiform encephalopathies, Creutzfeldt-Jakob Disease, government policy development, public health risk factors, Great Britian.

142. Jiang, XiuYun; Zhao, Quan; Ma, Hong Xia; Jiang, X.Y.; Quan, Z.; Ma, H.X. Mad cow disease and correlated disease with human CJD. Journal of Jilin Agricultural University. 2000. 22: 1, 91-95; 27 ref. ISSN: 1000-5684. In Chinese with an English summary.
     NAL call no. S19.C55
     Descriptors: BSE, bovine spongiform encephalopathy, Creutzfeldt Jakob disease, prion diseases, reviews.

143. Keulen, L.J.M. van.; Langeveld, J.P.M.; Garssen, G.J.; Jacobs, J.G.; Schreuder, B.E.C.; Smits, M.A. Diagnosis of bovine spongiform encephalopathy: A review. Vet Q. Utrecht, The Netherlands : Royal Netherlands Veterinary Association. Oct 2000. v. 22 (4) p. 197-200. ISSN: 0165-2176
     NAL call no. SF601.V46
     Descriptors: cows, bovine spongiform encephalopathy, diagnosis, symptoms, clinical aspects, animal behavior, brain, histopathology, postmortem examinations, detection, diagnostic techniques, evaluation, monitoring, literature reviews.

144. Killer conformations. Nature Structural Biology. 2000. v. 7 (9) p. 709-710
     Descriptors: prion protein, PrP, Nv Creutzfeldt Jakob disease, BSE, bovine spongiform encephalopathy, brain disease, food intake, degenerative disease, conformational transition, protein structure, human, nonhuman

145. Klein, R. The politics of risk: The case of BSE. British Medical Journal. Nov. 4, 2000. v. 321 (7269) p. 1091-1092
     NAL call no. R31 B55
     Descriptors: BSE, bovine spongiform encephalopathy, risk factor, health care policy, health hazards, cattle, Creutzfeldt Jakob disease, risk assessment, politics, health education, agriculture, human, editorial.

146. Knox, B. Consumer perception and understanding of risk from food. British Medical Bulletin. 2000. v. 56 (1) p. 97-109
     Abstract: The study of risk perception has been punctuated with controversy, conflict and paradigm shifts. Despite more than three decades of research, understanding of risk assessment remains fragmented and incoherent. Until recently, food and eating has been viewed as a low-risk activity and perceived risk surrounded matters of hygiene or lack of food. Consequently, theories of risk have been constructed with reference to environmental and technological hazards, such as nuclear power, whilst neglecting food issues. However, following a decade of 'food scares', attention has moved towards the study of food risk. Within this, food risk research has focused almost exclusively upon attempting to explain the divergence of opinion that exists between experts and the lay public whilst neglecting to address it. The following discussion provides a brief historical overview of theories and approaches that have been applied to the study of risk perception, continues with a summary of findings derived from food risk research and concludes with a discussion of methodological issues and some projections for future research.
     NAL call no. 448.2 B772
     Descriptors: BSE, consumer attitudes, risk assessment research, food risks, methods and theories

147. Korth, C.; Kaneko, K.; Heye, N.; Scott, M.; Prusiner, S.B. Transgenic mouse models for Creutzfeldt-Jakob disease. Society for Neuroscience Abstracts. 2000; 26 (1-2): Abstract No.-78.4. ISSN: 0190-5295. 30th Annual Meeting of the Society of Neuroscience, New Orleans, LA, USA, November 4-09, 2000
     NAL call no. QP351.S6
     Descriptors: chimeric MHu2M PrP transgene, wild-type mice, transgenic mice overexpressing mouse prion protein, species barriers, incubation times.

148. Krebs, J. Beefing about the risks posed by the French BSE epidemic. Nature. 2000 Dec 14; 408(6814): 767 ISSN: 0028-0836
     NAL call no. 472 N21
     Descriptors: BSE, bovine spongiform encephalopathy, France, letter.

149. Krebs, J.R. Science, uncertainty and policy: food for thought EUROTOX 2000, The XXXVIII European Congress of Toxicology, London (UK), 17-20 Sep 2000 Toxicology Letters (Boobis, -A.R.; Gooderham, N.; Hotchkiss, S.; Taehti, H.; Seiler, J.; Chipman, K.-eds.) 2000 vol. 116, no. 1-2, pp. 89-95 ISSN: 0378-4274
     NAL call no. RA1190.T62
     Abstract: The organisation and work of the Food Standards Agency are described. The Agency is a new non-Ministerial Government department with responsibility for protecting the health of the public and other interests of consumers in relation to food. Its roles encompass assessment of risk (through scientific expert committees) as well as risk communication and management. Among the many changes that the Agency has brought about is a new commitment to openness. All policy discussions and decisions take place in public. Diet and bovine spongiform encephalopathy are used as examples to illustrate the Agency's approaches to dealing with risk and uncertainty.
     Descriptors: food standards, government policy, public health risk-assessment, bovine spongiform encephalopathy British Isles.

150. Kretzschmar, H.A. Can BSE infection be transmitted through blood? That would be a real catastrophe. [Laast sich BSE durch blut ubertragen? Das ware wirklich eine katastrophe.] MMW-Fortschritte der Medizin. Sep. 28, 2000. v. 142 (39) p. 16
     Descriptors: BSE, bovine spongiform encephalopathy, etiology, disease transmission, blood products, Creutzfeldt Jakob disease, epidemiology, NvCreutzfeldt-Jakob disease, etiology, disease model, sheep, human, nonhuman.

151. Kretzschmar, H. Not only of interest in the differential diagnosis of Alzheimer's disease: BSE and other prion diseases. [Nicht nur als Differenzial diagnose der Alzheimer-Demenz interessant: BSE und andere Prionkrankheiten.] MMW Fortschritte der Medizin. 2000. v. 142 (39) p. 726-730
     Abstract: Prion diseases are rare neurodegenerative pathologies that are associated with dementia and various neurological deficits. They may present sporadically as familial or as infectious diseases. The most important differential diagnosis is Alzheimer's disease. Prion diseases have aroused particular interest ever since the appearance of bovine spongiform encephalopathy (mad cow disease), a prion disease of cattle that can apparently be transmitted to humans and which is the cause of a new variant of Creutzfeldt-Jakob Disease.
     Descriptors: BSE, bovine spongiform encephalopathy, human diseases, prion diseases, Creutzfeldt-Jakob Disease, man

152. Kretzschmar, H. The significance of prion diseases in the doctor's office setting. [BSE und andere prionkrankheiten.] MMW-Fortschritte der Medizin. Sep. 28, 2000. v. 142 (39) p. 34-38
     Descriptors: prion diseases, BSE, transmissible spongiform encephalopathies, transmission risks to humans, NvCreutzfeldt-Jakob Disease.

153. Krogsrud, Johan; Hopp, Petter; Bratberg, Bjorn; Ulvund, Martha J. Prion diseases - A general introduction. Norsk Veterinaertidsskrift. 2000. v. 112 (5) p. 321-329
     NAL call no. 41.8 N81
     Descriptors: prion diseases, transmissable encephalopathies, mink, cattle, sheep, cats, humans, Creutzfeldt-Jakob Syndrome, disease, bovine spongiform encephalopathy, chronic wasting disease, prion disease, feline spongiform encephalopathy, scrapie, transmissible mink encephalopathy, symptoms, comparisons, epidemiology.

154. Kumar, N.; Kukreti, S.; Ishaque, M.; Mulholland, R. Anatomy of deer spine and its comparison to the human spine. Anatomical record. Oct 1, 2000. 260(2): 189-203. ISSN: 0003-276X.
     Abstract: The anatomical parameters of the thoracic and lumbar regions of the deer spine were evaluated and compared with the existing data of the human spine. The objective was to create a database for the anatomical parameters of the deer spine, with a view to establish deer spine as a valid model for human spine biomechanical experiments in vitro. To date, the literature has supported the use of both calf and sheep spines as a suitable model for human spine experiments as the difficulty in procuring the human cadaveric spines is well appreciated. With the advent of Bovine Spongiform Encephalopathy (BSE) and its likely transmission to human in form of new variant Creutzfeld Jakob disease (CJD), there is a slight risk of transmission to humans through food chain if proper precautions for disposal of specimen are not adhered to. There is also a significant risk of transmission through direct inoculation to the researchers (Wells et al. Vet. Rec., 1998:142:103-106), working with infected bovine and sheep spine. The deer spines are readily available and there are no reported cases of deer being carriers of prion diseases (Ministry of Agriculture, Fisheries and Food, 1998). Six complete deer spines were measured to determine 22 dimensions from the vertebral bodies, endplates, disc, pedicles, spinal canal, transverse and spinous processes, articular facets. This was compared with the existing data of the human spine in the literature. The deer and human vertebrae show many similarities in the lower thoracic and upper lumbar spine, although they show substantial differences in certain dimensions. The cervical spine was markedly different in comparison. The deer spine may represent a suitable model for human experiments related to gross anatomy of the thoracic and lumbar spine. A thorough database has been provided for deciding the validity of deer spine as a model for the human spine biomechanical in vitro experiments.
     NAL call no. 447.8 AN1
     Descriptors: deer spine anatomy, model for human spine, BSE risks from bovine based materials, thoracic and lumbar regions.

155. Lang, N.P.; Hammerle, C.; Oesch, B.; Schenk, R.K.; Honig, J.F.; Metten, H.A. Risk of transmission of agents associated with Creutzfeldt-Jakob Disease and bovine spongiform encephalopathy. Plastic and Reconstructive Surgery. 2000. v. 105 (6) p. 2273-2275
     Descriptors: BSE, Creutzfeldt Jakob disease, bovine spongiform encephalopathy, medical device safety, disease transmission risks, bone prosthesis.

156. Lasmezas, C. I.; Weiss, S.; Cary, J. W.; Linz, J. E.; Bhatnagar, D. Molecular biology of prion diseases. Microbial foodborne diseases: mechanisms of pathogenesis and toxin synthesis. Lancaster, [PA?]: Technomic Publishing Company, Inc., 2000, 9 refs. ISBN: 1-56676-787-3.
     NAL call no. QR201 F56 M53 2000
     Descriptors: molecular biology, prion diseases, Creutzfeldt-Jakob Disease, bovine spongiform encephalopathy, BSE.

157. Latouche, K.; Rainelli, P.; Vermersch, D. Which price for food security? Contingent valuation following the European crisis of "mad cow". Can J Agri Econ. Ottawa : Canadian Agricultural Economics and Farm Management Society,. Nov 2000. v. 48 (3) p. 325-340. ISSN: 0008-3976. French with a summary in English.
     NAL call no. 281.8 C16
     Descriptors: beef, consumer behavior, bovine spongiform encephalopathy, agricultural crises, food and nutrition controversies, consumer attitudes, econometric models, Creutzfeldt-Jakob Disease, Brittany.

158. Lemaire-Vieille, C.; Schulze, T.; Podevin-Dimster, V.; Follet, J.; Bailly, Y.; Blanquet-Grossard, F.; Decavel, J. P.; Heinen, E.; Cesbron, J. Y. Epithelial and endothelial expression of the green fluorescent protein reporter gene under the control of bovine prion protein (PrP) gene regulatory sequences in transgenic mice. Proceedings of the National Academy of Sciences of the United States of America. 2000. v. 97 (10) p. 5422-5427, 48 refs ISSN: 0027-8424
     NAL call no. 500 N21P
     Descriptors: prion proteins, PrPc, regulatory sequences, transgenic animals, alternative splicing, brain, gene expression, scrapie, bovine spongiform encephalopathy, green fluorescent protein reporter gene, cellular distribution, transgenic mice model.

159. Leon, S. F. E; Rodriguez, C. I; Prada, D. G. Prions, infections and confusion in the "transmissible" spongiform encephalopathies. The evidence-based science III. Review. [Priones, infecciones y confusiones en las encefalopatias espongiformes "trasmisibles". Ciencia basada en la otra evidencia III. Revision.] Investigacion Clinica. 2000. v. 41 (3) p. 189-210.
     Abstract: There are some neurological disorders with a pathological hallmark called spongiosis which include Creutzfeld-Jakob disease and its new variant, the Gertsmann-Straussler-Scheinker Syndrome and the Fatal Familial Insomnia in humans; and Scrapie and Bovine Spongiform Encephalopathy, among others, in animals. The etiological agent has been considered either transmissible or hereditary or both. Curiously, this agent has no nucleic acids, is impossible to filter, is resistant to inactivation by chemical means, has not been cultured and is unobservable at electron microscopy. All of these facts have led to some researches to claim that these agents are similar to viruses appearing in computers. However, after almost fifty years of research, is still not possible to explain why and how such elements produce the diseases commented about. On the contrary, during these years have been possible to know that these entities called slow viral infections, transmissible amyloidosis, transmissible dementia, transmissible spongiform encephalopathies or prion diseases appear in individuals with genetical predispositions exposed to several worldwide immunological stressors. The possibility that prions are the consequence and not the cause of these diseases in animals and man is day by day more reliable, and supports the suggestion that a systematic intoxication due to pesticides as well as mycotoxin ingestion, produced mainly by different molds such as Aspergillus, Penicillium or Fusarium, seem to be the true etiology of these neurodegenerative disorders.
     Descriptors: humans, sheep, cattle, NvCeutzfeldt-Jakob Disease, Gertsman-Straussler-Scheinker Syndrome, fatal familial insomnia, scrapie, BSE, bovine spongiform encephalopathy, etiology, prions, possible cause of infection, pesticides, mycotoxins, review article.

160. Lessons for the future. The Veterinary record: journal of the British Veterinary Association. London: The British Veterinary Association. Oct. 28, 2000. v. 147 (18) p. 493.
     NAL call no. 41.8 V641
     Descriptors: bovine spongiform encephalopathy, Creutzfeldt-Jakob Disease, disease control, reports, United Kingdom.

161. Liberski, P.P. Bovine spongiform encephalopathy and variant Creutzfeldt-Jakob disease: a risk analysis. Folia-Neuropathologica. 2000. 38(4): 143-150
     Abstract: There is doubt that variant Creutzfeldt-Jakob disease (vCJD) resulted from bovine spongiform encephalopathy (BSE) transmission from cattle to human. What is uncertain is the total number of vCJD cases (currently about 80). In this review I covered recent data on the vCJD and BSE epidemic, the mode of BSE spreading to humans and, finally, the data on the PRNP analogue--the doppel gene (PRND).
     Descriptors: NvCreutzfeldt-Jakob Disease, BSE

162. Lopez Garcia, F.; Zahn, R.; Riek, R.; Wuthrich, K. NMR structure of the bovine prion protein. Proc Natl Acad Sci USA. Jul 18, 2000. 97(15): 8334-8339. ISSN: 0027-8424.
     Abstract: The NMR structures of the recombinant 217-residue polypeptide chain of the mature bovine prion protein, bPrP(23-230), and a C-terminal fragment, bPrP(121-230), include a globular domain extending from residue 125 to residue 227, a short flexible chain end of residues 228-230, and an N-terminal flexibly disordered "tail" comprising 108 residues for the intact protein and 4 residues for bPrP(121-230), respectively. The globular domain contains three alpha-helices comprising the residues 144-154, 173-194, and 200-226, and a short antiparallel beta-sheet comprising the residues 128-131 and 161-164. The best-defined parts of the globular domain are the central portions of the helices 2 and 3, which are linked by the only disulfide bond in bPrP. Significantly increased disorder and mobility is observed for helix 1, the loop 166-172 leading from the beta-strand 2 to helix 2, the end of helix 2 and the following loop, and the last turn of helix 3. Although there are characteristic local differences relative to the conformations of the murine and Syrian hamster prion proteins, the bPrP structure is essentially identical to that of the human prion protein. On the other hand, there are differences between bovine and human PrP in the surface distribution of electrostatic charges, which then appears to be the principal structural feature of the "healthy" PrP form that might affect the stringency of the species barrier for transmission of prion diseases between humans and cattle.
     NAL call no. 500 N21P
     Descriptors: bovine prion protein, PrP, human prion protein, structure comparison.

163. Love, S.; Helps, C. R.; Williams, S.; Shand, A.; McKinstry, J. L.; Brown, S. N.; Harbour, D. A.; Anil, M. H. Methods for detection of haematogenous dissemination of brain tissue after stunning of cattle with captive bolt guns. Journal of Neuroscience Methods. June 30, 2000. v. 99 (1-2) p. 53-58.
     Abstract: Because of concern that the stunning of cattle with captive bolt guns (CBGs) could, if used on an animal with bovine spongiform encephalopathy (BSE), cause embolism of infective brain tissue and carcass contamination, the Ministry of Agriculture, Food and Fisheries commissioned research to assess the risk of haematogenous dissemination of CNS material after stunning. We have devised two methods to investigate this risk. The first involves the concentration of embolic tissue in buffy coat Cytoblocks that can be embedded for sectioning, microscopy and immunocytochemistry. The second method is an ELISA for the presynaptic protein, syntaxin 1B. The methods were validated by analysis of several bovine tissues, including blood samples deliberately contaminated with brain. We then studied jugular venous blood obtained before and after the stunning of 60 cattle with CBGs. Samples obtained, after stunning, from five of the cattle contained CNS tissue within the Cytoblocks and yielded positive syntaxin assays. Syntaxin was also detected in samples from one other animal that had been stunned with a pneumatically operated CBG. The described methods should allow an assessment of the risk of neuroembolism associated with different types of CBG and may also be useful in other contexts.
     Descriptors: Ministry of Agriculture, Food and Fisheries, BSE, cattle, post captive bolt stunning, risk of disease agent dissemination, ELISA, embolic tissue in buffy coat Cytoblocks, syntaxin assays, jugular venous blood sampling, UK

164. Machado, M.; Tavares, A.; Ramos, M.; Carvalho, C. Ocorrencia de Listeria monocytogenes e de outras especies de Listeria em cerebros de bovinos suspeitos de Encefalopatia Espongiforme dos Bovinos. [Occurrence of Listeria monocytogenes and other Listeria spp. in brains of cattle with suspected BSE.] Veterinaria Tecnica. 2000, 10: 3, 30-33; 18 ref. In Portuguese with an English summary.
     Descriptors: brain diseases, encephalitis, histopathology, meningitis, Listeria monocytogenes, meningoencephalitis, pathogenecity

165. Madec, J.Y.; Belli, P.; Calavas, D.; Baron, H. Efficiency of Western blotting for the specific immunodetection of proteinase K-resistant prion protein in BSE diagnosis in France. The Veterinary record: journal of the British Veterinary Association. London: The British Veterinary Association. Jan. 15, 2000. v. 146 (3) p. 74-76. Includes references
     NAL call no. 41.8 V641
     Descriptors: cattle, bovine spongiform encephalopathy, prion proteins, western blotting, immunodiagnosis, brain, histopathology, France.

166. Madec, J.; Groschup, M.H.; Calavas, D.; Junghans, F.; Baron, T. Protease-resistant prion protein in brain and lymphoid organs of sheep within a naturally scrapie-infected flock Microbial Pathogenesis 2000 vol. 28, no. 6, pp. 353-362 ISSN: 0882-4010.
     NAL call no. QR175.M53
     Descriptors: transmissible spongiform encephalopathies, PrPres, prion protein, scrapie, PrP genotypes, assays of various body organs, brain stem, cerebellum, VRQ allele, cattle, BSE, glycopattern.

167. Mahy, B.W.J.; Brown, C. C. Emerging zoonoses: Crossing the species barrier. Revue Scientifique et Technique Office International des Epizooties. April 2000. v. 19 (1) p. 33-40.
     Abstract: The ability of infectious disease agents to cross the species barrier has long been recognised for many zoonotic diseases. New viral zoonotic diseases, such as acquired immune deficiency syndrome (AIDS). Influenza virus continues to move from avian species into humans. The filoviruses and the newer paramyxoviruses, Hendra and Nipah, highlight the increasing proclivity of some animal viral agents to infect human populations with serious diseases. A transmissible spongiform encephalopathy, bovine spongiform encephalopathy, has emerged in cattle in Europe and spread to humans and other animal species. A novel toxicosis, caused by Pfiesteria spp., has become a secondary problem in some areas where large fish kills have occurred. The increasing proximity of human and animal populations has led to the emergence of, or increase in, bacterial zoonoses such as plague, leptospirosis and ehrlichiosis. The factors which influence the ability of each infectious agent to effectively cross the species barrier are poorly understood. However, for all of these diseases, the underlying theme is the growth of the human population, the mobility of that population, and the food production efforts.
     NAL call no. SF781 R4
     Descriptors: zoonotic diseases, crossing species barriers, filoviruses, paramyxoviruses, Hendra, Nipah, transmissible spongiform encephalopathies, BSE, bovine spongiform encephalopathy, Pfiesteria, bacterial diseases, plague, leptospirosis and ehrlichiosis, human population pressures.

168. Manousis, T.; Verghese-Nikolakaki, S.; Keyes, P.; Sachsamanoglou, M.; Dawson, M.; Papadopoulos, O.; Sklaviadis, T. K. Characterization of the murine BSE infectious agent. Journal of General Virology. 2000. v. 81 (6) p. 1615-1620, 26 refs. ISSN: 0022-1317
     Abstract: Bovine spongiform encephalopathy (BSE) is a prion-associated disease where the infectious agent is thought to be a host-encoded protein with a protease-resistant conformation (PrP(Sc)). Here, data are presented on the solubilization of purified murine BSE material, using guanidine-HCl as a denaturing agent. This treatment led to loss of infectivity, which was partially recovered on renaturation after dialysis to remove the chaotropic agent. The renatured product was then fractionated on an isopycnic sucrose-density gradient and the fractions were analysed for the presence of PrP(Sc), nucleic acids and infectivity. It was found that the major part of PrP(Sc) (>90%) and the endogenous nucleic acids did not contribute towards the formation of infectious particles on renaturation. Infectivity was distributed in the top three, low-density fractions. Among these, the presence of considerable infectivity in the fraction of lowest density, with barely detectable PrP(Sc), is of particular interest.
     NAL call no. QR360.A1J6
     Descriptors: bovine spongiform encephalopathy, BSE, prion disease, prion proteins, PrPSc, guanidine-HCl denaturing agent, mouse tissue, infectivity testing.

169. Manuelidis, Laura; Lu, Zhi Yun. Attenuated Creutzfeldt-Jakob Disease agents can hide more virulent infections. Neuroscience Letters. November 3, 2000. v. 293 (3) p. 163-166.
     Abstract: We previously showed that a slow infectious strain of Creutzfeldt-Jakob Disease (CJD) can dramatically suppress the expression of a fast virulent agent injected intracerebrally 80 days later. While the slow SY agent eventually produced disease at apprx 400 days, there was little evidence of the fast FU agent. However, two of 18 superinfected mice showed a minor increase in pathologic changes. All traces of FU were obliterated in typical brains of suppressed mice. The two aberrant mice however had mixed SY and FU infections, with FU reappearing at late stages of SY disease. Thus less virulent sporadic CJD infections in older people can conceal other agents such as variant CJD, the more recently evolved and virulent agent linked to bovine spongiform encephalopathy. This powerful model of agent-induced repression also implicates targets other than prion protein (PrP) in eliminating infection.
     NAL call no. QP351 N3
     Descriptors: BSE, mouse model, prion disease, laboratory infection, Creutzfeldt-Jakob Disease, bovine spongiform encephalopathy

170. Marwick, C. FDA calls bovine-based vaccines currently safe. JAMA (Journal of the American Medical Association) Sep 13, 2000. 284(10): 1231-1232. ISSN: 0098-7484
     NAL call no. 448.9 Am37
     Descriptors: Vaccines, BSE, fetal calf serum, contamination risks, US Food and Drug Administration, risks from imported sources, NvCreutzfeldt-Jakob Disease, advisory committee recommendations, guidance notices

171. Mazzocchi, M. Crises et changements structurels dans la consommation alimentaire: un systeme structurel de demande. [Shocks and structural change in food consumption: structural demand system.] Cahiers d'Economie et Sociologie Rurales. 2000, No. 54, 27-46; 47 ref. ISSN: 0755-9208. In French with an English summary.
     Descriptors: modeling, consumer food habits, impact of food safety information, BSE, Italy, meat demand post BSE scare, the time-varying Marshallian own-price elasticity and the expenditure elasticity, time pattern of food consumption.

172. Meere, F. B. J. de Sepers, C. A wrong image? Newspaper and press messages during the BSE crisis. [Een verwrongen beeld? Kranten- en persberichten tijdens de BSE-crisis.] Rapport - Landbouw-Economisch Instituut (LEI). Netherlands: Den Haag, 2000. (No. 5.00.03). 36 pp. 6 refs. ISBN: 90-5242-578-7.
     NAL call no. 280.9 L23
     Descriptors: newspapers, bovine spongiform encephalopathy, diffusion of information, journalism, public opinion, Netherlands.

173. Meslin, F. X.; Stohr, K.; Heymann, D. Public health implications of emerging zoonosis. Revue Scientifique et Technique Office International des Epizooties. April 2000. v. 19 (1) p. 310-317
     NAL call no. SF781 R4
     Descriptors: zoonotic diseases, direct effects, indirect effects, health and food policies, international cooperation, Ebola virus, avian influenza, monkeypox and bovine spongiform encephalopathy.

174. Miller, M.W.; Williams, E.S.; McCarty, C.W.; Spraker, T.R.; Kreeger, T.J.; Larsen, C.T.; Thorne, E.T. Epizootiology of chronic wasting disease in free-ranging cervids in Colorado and Wyoming. J Wildl Dis. Lawrence, Kan. : Wildlife Disease Association Inc. Oct 2000. v. 36 (4) p. 676-690. ISSN: 0090-3558
     Abstract: Surveillance and epidemic modeling were used to study chronic wasting disease (CWD), a transmissible spongiform encephalopathy that occurs naturally among sympatric, free-ranging deer (Odocoileus spp.) and Rocky Mountain elk (Cervus elaphus nelsoni) populations in contiguous portions of northeastern Colorado and southeastern Wyoming (USA). We used clinical case submissions to identify endemic areas, then used immunohistochemistry to detect CWD-infected individuals among 5,513 deer and elk sampled via geographically-focused random surveys. Estimated overall prevalence (prevalence, 95% confidence interval) in mule deer (4.9%, 4.1 to 5.7%) was higher than in white-tailed deer (2.1%, 0.5 to 3.4%) or elk (0.5%, 0.001 to 1%) in endemic areas; CWD was not detected in outlying portions of either state. Within species, CWD prevalence varied widely among biologically- or geographically-segregated subpopulations within the 38,137 km2 endemic area but appeared stable over a 3-yr period. The number of clinical CWD cases submitted from an area was a poor predictor of local CWD prevalence, and prevalence was typically > or =1% before clinical cases were first detected in most areas. Under plausible transmission assumptions that mimicked field data, prevalence in epidemic models reached about 1% in 15 to 20 yr and about 15% in 37 to 50 yr. Models forecast population declines once prevalence exceeded about 5%. Both field and model data supported the importance of lateral transmission in CWD dynamics. Based on prevalence, spatial distribution, and modeling, we suggest CWD has been occurring in northeastern Colorado and southeastern Wyoming for >30 yr, and may be best represented as an epizootic with a protracted time-scale.
     NAL call no. 41.9 W64B
     Descriptors: deer, elk, Odocoileus hemionus, Odocoileus virginianus, Cervus elaphus canadensis, prion diseases, spongiform encephalopathy, epidemiology, Colorado, Wyoming.

175. Millstone, E.; Van Zwanenberg, P. A crisis of trust: For science, scientists or for institutions? Nature Medicine. 2000, v. 6 (12) p. 1307-1308.
     Descriptors: stem cells, xenotransplantation, biotechnology issues, medical research, BSE, social issues.

176. Minor, P.D.; Will, R.G.; Salisbury, D. Vaccines and variant CJD Vaccine 2000 vol. 19, no. 4-5, pp. 409-410 ISSN: 0264-410X
     NAL call no. QR189.V32
     Descriptors: NvCreutzfeldt-Jakob disease, bovine-based products, contamination, vaccines, human health risks.

177. Molenda, Jerzy. A new variant of Creutzfeldt-Jakob Disease and bovine spongiform encephalopathy. Medycyna Weterynaryjna. 2000. v. 56 (6) p. 355-362
     Abstract: In March 1996 the UK government stated that the most likely cause of a new disease of defined as being a new variant of Creutzfeldt-Jakob Disease (vCJD) was exposure to a causative agent of bovine spongiform encephalopathy (BSE). Additional cases and research point to a link between these diseases. Researchers led by Moira Bruce and John Collinge reinforce the conclusion that vCJD is quite distinct from other forms of CJD. Utilizing a mouse transmission model, they provide essential data indicating that this new variant of the disease is caused by an agent strain of BSE. The article discusses the data which both confirm and question this conclusion.
     NAL call no. 41.8 M463
     Descriptors: bovine spongiform encephalopathy, NvCreutzfeldt-Jakob Disease, prion diseases, variant strains, transmissability, mouse model.

178. Momcilovic, Dragan; Rasooly, Avraham. Detection and analysis of animal materials in food and feed. Journal of Food Protection. November, 2000. v. 63 (11) p. 1602-1609
     Abstract: Bovine spongiform encephalopathy (BSE) belongs to a group of progressively degenerative neurological diseases known as transmissible spongiform encephalopathies (TSEs) associated with a variant form of Creutzfeldt-Jakob Disease in humans. TSEs are fatal diseases caused by prions (proteinaceous infectious particle) and are characterized by an incubation period that may range from several months to several years, depending on the host. Because BSE is spread through animal feed, the main strategy for preventing the establishment and spread of BSE is to prohibit the use of proteins derived from mammalian tissue in feed for ruminant animals. Enforcement of these regulations relies on the ability to identify the presence of prohibited proteins in ruminant feed. The methods to detect bovine products in feed materials are based on analyses of DNA, bone, or protein. In this article, discusses the current methodology as well as other potentially useful methods of analysis of animal material in food. No methods specifically distinguish between prohibited bovine material and allowable bovine products, such as milk or blood. Furthermore, all these methods are hampered by the fact that the rendering process involves heat treatment that denatures and degrades proteins and DNA. There is a need for improving existing methods and developing new methods to overcome these two limitations.
     NAL call no. 44.8 J824
     Descriptors: feed and food analysis, prion contamination, methods, bovine spongiform encephalopathy, Creutzfeldt-Jakob Syndrome.

179. Moorby, J.M.; Dhanoa, M.S.; Austin, A.R. Aspects of the metabolism of dairy cows during the incubation of bovine spongiform encephalopathy. The Veterinary record: journal of the British Veterinary Association. London: The British Veterinary Association. Oct. 7, 2000. v. 147 (15) p. 409-412. Includes references
     Abstract: As part of a nutritional study lasting from six weeks before calving to 22 weeks of lactation, blood samples collected from 47 dairy cows maintained under well-defined conditions were analysed for a variety of metabolites and hormones. At various times after the completion of the study, six of the animals developed clinical signs of bovine spongiform encephalopathy (BSE), although when they were sampled it was not known that they were incubating the disease. The data were used to make comparisons between the animals that developed BSE and those that did not develop the disease and which had been maintained under the same conditions. The greatest differences between the animals incubating BSE and the control animals were observed at times of nutritional stress, at the start of lactation and when the intake of concentrate feeds was reduced at week 13 of lactation. In the animals that subsequently developed BSE, feed intakes were lower in early lactation; plasma beta-hydroxbutyrate concentrations were significantly higher (P<0.001) at weeks 3 and 5 of lactation; adjusted milk yields were lower until week 6 of lactation and milk fat concentrations were consistently lower. There was no effect on plasma glucose concentrations, although insulin concentrations were significantly lower in week 1 of lactation (2-27 v 2.50 microiu/ml) (P<0.05). The concentrations of plasma proteins and urea were unaffected by BSE incubation, apart from protein concentrations being significantly higher one week before calving, and the concentration of urea being significantly lower five weeks before calving. The plasma concentrations of somatotrophin, prolactin, oestradiol and progesterone were similar in the two groups of animals throughout the study. The differences observed indicate that the energy metabolism of dairy cows incubating BSE may be subtly altered before the onset of clinical signs of the disease.
     NAL call no. 41.8 V641
     Descriptors: dairy cows, bovine spongiform encephalopathy, energy metabolism, nitrogen metabolism, prepatent period, feed intake, metabolites, hormones, body condition, liveweight, milk yield.

180. Nailon, W.H,; Ironside J.W. Variant Creutzfeldt-Jakob Disease: Immunocytochemical studies and image analysis. Microscopy Research and Technique. July 1, 2000. v. 50 (1) p. 2-9
     Abstract: Variant Creutzfeldt-Jakob disease (vCJD) is a recently identified human prion disease that appears to arise from exposure to the bovine spongiform encephalopathy agent. The clinical features and neuropathology of vCJD are distinctive, particularly the patterns of PrP(sc) accumulation in the brain. PrP immunocytochemistry has also demonstrated the accumulation of PrP(sc) in tissues outside the central nervous system, including sensory ganglia and lymphoid tissues. These observations have allowed the use of tonsillar biopsy as an investigation to aid the diagnosis of vCJD, since accumulation of PrP(sc) in lymphoid tissues does not occur in other forms of human prion disease. The patterns of PrP(sc) accumulation in vCJD can be studied by image analysis techniques, using both quantitative and qualitative approaches. Preliminary results of textural analysis are presented, which indicate that this approach can be used to discriminate and study the unique features of PrP(sc) accumulation in the brain in vCJD. This technique has major potential as a research tool in human prion diseases, particularly for the characterisation of disease phenotype in large series of cases.
     NAL call no. QH212 E4J69
     Descriptors: NvCreutzfeldt-Jakob Disease, human prion diseases, BSE, bovine spongiform encephalopathy agent, PrP(sc) accumulation in nervous and lymphoid tissues, tonsillar biopsy.

181. Nakamura, N.; Aoki, Y.; Horiuchi, H.; Furusawa, S.; Yamanaka, H.I.; Kitamoto, T.; Matsuda, H. Construction of recombinant monoclonal antibodies from a chicken hybridoma line secreting specific antibody. Cytotechnology. 2000. v. 32 (3) p. 191-198, 26 refs.
     NAL call no. QH585 C97
     Descriptors: poultry, monoclonal antibodies, hybridomas, bovine spongiform encephalopathy, ELISA, prions.

182. Niemer, U. To date, there has been no case of BSE in Germany. We must keep up our guard. [Bisher noch keine BSE-erkrankungen in Deutschland: wir mussen weiter wachsam bleiben.] MMW-Fortschritte der Medizin. Sep. 28, 2000. v. 142 (39) p. 33
     Descriptors: BSE, bovine spongiform encephalopathy, etiology, Nv Creutzfeldt-Jakob disease, epidemiology, disease prevention, epidemiological data, monitoring, preventive medicine, human, nonhuman, Germany.

183. Norrung, B.; Willeberg, P. Kogalskab/bovin spongiform encefalopati. [Mad cow disease/bovine spongiform encephalopathy] Ugeskrift for Laeger. 2000 Aug 21; 162(34): 4518-9 ISSN: 0041-5782. In Danish.
     Descriptors: BSE, bovine spongiform encephalopathy, prion diseases

184. Novak, M.; Vrtiak, O. J.; Mikula, I.; Tkacikova, L. Ovine scrapie: priorities and importance. Folia microbiologica. 2000. 45(6): 475-483. ISSN: 0015-5632.
     Abstract: Ovine and caprine scrapie occupies a unique place among animal transmissive spongiform encephalopathies (TSE). It is an object of intensive biomedicinal, ecological and economical studies. Its causative agents are demonstrably associated with the development of TSE in farmed minks, goats and moufflons. Ovine strains of scrapie occurring in North America (particularly in the USA) differ from strains which occur in Europe and were present at the onset of development of TSE in three species of deer living in free nature and in captivity in the USA. The studies dealing with the development of bovine spongiform encephalopathy (BSE) of the English type have indicated justifiably that its origin is associated with one (or more) heretofore unidentified ovine strain. The development of a variant form, the Creutzfeldt-Jacob disease in humans, and transmission of the BSE agent to several families of bovidae, felidae and primates, puts stress on its zoonotic potential. All this leads to the conclusion that domesticated sheep are the decisive reservoir species of animal TSE. They have been infected to an unknown extent with the causative agent of BSE probably through contaminated meat-bone meal. The occurrence of natural ovine prion isolates with properties similar to those of the BSE agent requires that scrapie should be included in the surveillance of human and animal TSE. At present, scrapie is a noticeable disease also in other than European Communities Member States. It is on the list B of the International Epizootics Office. Many countries have initiated control of ovine scrapie. It should therefore become a topical question also in Central and Eastern European countries. Elimination or even eradication of ovine scrapie (or its causative agents) from populations of small and large domestic ruminants is the prerequisite for prevention of penetration of ovine pathogenic prions into the human feed chain. Moreover, it should be ensured that these species will be able to produce foods of a new type (immunotrition and similar) or proteins with therapeutic effects in the near future. Our study established that the PrP genotype of Valachian rams, the Slovak autochthonous breed, contains also VRQ and ARQ alleles encoding the susceptibility to scrapie. Their selection is part of the improvement of Slovak Valachian sheep towards resistance to scrapie.
     NAL call no. 448.3 C332
     Descriptors: scrapie, sheep, TSEs, BSE, role of sheep as a reservoir, EU, listing in IEO, elimination, eradication, PrP genotype, Slovak sheep breed.

185. O'Brien, M. Have lessons been learned from the UK bovine spongiform encephalopathy (BSE) epidemic? International journal of epidemiology. Aug 2000. 29(4): 730-733. ISSN: 0300-5771.
     Abstract: History is full of examples of public health, commerce and politics in conflict. In recent years attempts to protect UK egg producers, after the discovery of Salmonella enteritidis (phage type 4) in hens' eggs, strained previously good working relationships between medical and veterinary epidemiologists and ended the political career of a government minister who spoke out in defence of the public health. Against the background lessons of earlier high profile public health problems in the UK conflict should have been avoided when bovine spongiform encephalopathy (BSE) started. It might have been expected that its significance for human health could have been recognized and researched earlier. Public announcements about it could have been timely and clear. Unfortunately this was not the case and it looks as though similar mistakes are going to be repeated over genetically modified foods.
     Descriptors: BSE, comment on handling of epidemic, health risk management.

186. Oesch, B.; Doherr, M.; Heim, D.; Fischer, K.; Egli, S.; Bolliger, S.; Biffiger, K.; Schaller, O.; Vandevelde, M.; Moser, M. Application of Prionics Western blotting procedure to screen for BSE in cattle regularly slaughtered at Swiss abattoirs. Archives of virology. Supplementum. 2000. (16): 189-195. ISSN: 0939-1983.
     Abstract: Disease-specific PrP (PrP(Sc)) is at least part of the infectious particle (prion) causing bovine spongiform encephalopathy (BSE) or scrapie in sheep. Digestion with protease allows a distinction between normal PrP (PrP(C)) and PrP(Sc) i.e. PrP(C) is completely digested while PrP(Sc) is cleaved at the N-terminus leading to a fragment of reduced molecular weight (PrP 27-30). Detection of this fragment by Western blotting has been described more than a decade ago for rodent PrP. We have now optimized the technique in order to allow rapid analysis of hundreds of samples per day. Here we report the application of this technique to the analysis of 3000 regularly slaughtered cattle from Swiss abattoirs. For comparison all the animals were subsequently examined by classical methods (i.e. histology and immunohistochemistry). All but one animal were negative for BSE by all methods. The Western blot positive animal was confirmed to be a BSE case and the carcass was removed from the food chain. We conclude that it is feasible to examine slaughtered cattle on a routine basis without causing delays to the meat processing industry.
     NAL call no. QR355 A72
     Descriptors: BSE, cattle diagnostic test, post slaughter examination, Prionics Western blot, Switzerland, validation.

187. Of BSE, surveillance, and the veterinary surgeon's privilege to dispense. Veterinary Record 2000 Oct 7; 147(15): 403-4 ISSN: 0042-4900
     NAL call no. 41.8 V641
     Descriptors: British Veterinary Association, summary of president's remarks, BSE inquiry, crisis decision making, surveillance.

188. Office International des Epizooties. Bovine spongiform encephalopathy in Spain, Germany and Uruguay; Foot and mouth disease in Swaziland; Newcastle disease in Mexico. Disease Information Office International des Epizooties. 2000. 13: 47, 215-219. ISSN: 1012-5329
     NAL call no. SF781 D57
     Descriptors: BSE, bovine spongiform encephalopathy, foot and mouth disease, Newcastle disease, Spain, Uruguay, Swaziland, Mexico, disease incidence.

189. Office International des Epizooties. World animal health in 1999. Part 1: reports on the animal health status and disease control methods and tables on incidence of list A diseases. 2000, 339 pp.; ISSN 1017-3102. Published by OIE, 12 rue de Prony.; 75017 Paris; France
     Descriptors: animal diseases, livestock, disease statistics, various important animal diseases, BSE, Office International des Epizooties, World.

190. Olah, G.A. From scrapie to prions: veterinarians pave the way. Veterinary Heritage. 2000, 23: 2, 38-42; 21 ref.
     NAL call no. SF615 V472
     Descriptors: scrapie, veterinary history, veterinarians, prion diseases, transmissible spongiform encephalopathies, BSE.

191. Onodera, Takashi; Saeki, Keiichi. Japanese scrapie cases. Japanese Journal of Infectious Diseases. April 2000. v. 53 (2) p. 56-61
     Abstract: Worldwide attention has been given to scrapie, because bovine spongiform encephalopathy (BSE) could be experimentally transmitted to sheep. This ovine form of BSE was clinically identical to scrapie. In Japanese scrapie cases, a majority of the diseased sheep were from the Suffolk breed, while 8 cases were from Corriedale breed. It is very likely that sheep-to-sheep transmission of scrapie has taken place in Obihiro, Hokkaido. Normal prion protein may play a role in the morphoregulatory signaling pathway. Over-expression of normal prion protein in mice causes neurodegenerative disorders. Recently, Prnd was identified downstream of the mouse prion protein gene (Prnp), and encodes 179 amino acids and a prion protein (PrP)-like protein designated doppel (Dpl). Dpl was upregulated in the central nervous system of two PrP-deficient lines of mice, as well as in prionless cell lines. Dpl caused neurodegeneration similar to that caused by PrP. Linked expression of Prnp and Prnd may cause several neurodegenerative disorders.
     Descriptors: BSE, bovine spongiform encephalopathy, prion disease, scrapie, sheep-to-sheep transmission, PrP, doppel protein, mouse Prnp gene, mouse protein prion gene.

192. Orge, L.; Simas, J.P.; Fernandes, A.C.; Ramso, M.; Galo, A. Similarity of the lesion profile of Bovine spongiforme [sic] encephalopathy in France--few numbers and data: appendix. [Paris?]: Republic of France, Ministry of Agriculture and Fisheries, General Directorate for Food, [2000]. Chiefly tables "Updated: 02/08/00."
     NAL call no. SF967.S63 B6842 2000
     Descriptors: BSE, bovine spongiform encephalopathy, animal diseases, food safety, France.

193. Orge, L.; Simas, J.P.; Fernandes, A.C.; Ramso, M.; Galo, A. Similarity of the lesion profile of BSE in Portuguese cattle to that described in British cattle. The Veterinary record: journal of the British Veterinary Association. London: The British Veterinary Association. Oct. 21, 2000. v. 147 (17) p. 486-488. Includes references.
     NAL call no. 41.8 V641
     Descriptors: cattle, bovine spongiform encephalopathy, lesions, central nervous system, histopathology, Portugal, United Kingdom.

194. Painter, M.J. Variant Creutzfeldt-Jakob disease. Journal of Infection. 2000. v. 41 (2) p. 117-124.
     Descriptors: BSE, NvCreutzfeldt-Jakob Disease, PrP, prion protein, drinking water, disease transmission, bovine spongiform encephalopathy, scrapie, exposure, cattle, infection, inoculation, sexual transmission, infection control, prediction, sheep, treatment planning, ingestion, inhalation, public health risks, UK, human, nonhuman, controlled study, review.

195. Parren, P.W.H.I.; Poignard, P.; Ditzel, H.J.; Williamson, R.A.; Burton, D.R. Antibodies in human infectious disease. Immunologic Research. 2000. v. 21 (2-3) p. 265-278
     NAL call no. QR180 S88
     Descriptors: PrP, prion proteins, antibodies, molecular events of pathology, human immunodeficiency virus 1, Ebola virus, respiratory syncytial pneumovirus, prion disease, Creutzfeldt-Jakob disease, etiology, BSE, bovine spongiform encephalopathy

196. Pascual, F. The future of beef and its labelling. [El futuro de la carne de vacuno y su etiquetado.] Mundo Ganadero. 2000. v. 11 (119) p. 36-41.
     Descriptors: EU beef, BSE, labeling, EU standards, meat products, animal health, consumer confidence, quality assurance.

197. Past lessons, future concerns. Vet Rec. London : The British Veterinary Association. Dec 23/30, 2000. v. 147 (26) p. 725-726. ISSN: 0042-4900
     NAL call no. 41.8 V641
     Descriptors: bovine spongiform encephalopathy, food safety, meat and livestock industry, UK.

198. Peano, S.; Reiner, G.; Carbonatto, M.; Bodenbender, L.; Boland, P.; Abel, K. J. Determination of the clearance factor for transmissible spongiform encephalopathy agents during the manufacturing process of polygeline. Intensive Care Medicine. 2000. v. 26 (5) p. 608-612
     Abstract: OBJECTIVE: To determine the safety of polygeline, a gelatine-derived plasma substitute produced from bovine bones, in terms of safety for bovine spongiform encephalopathy (BSE) by evaluating the ability of the manufacturing process of polygeline to eliminate agents related to transmissible spongiform encephalopathy (TSE) through the validation of three main production steps. DESIGN: Laboratory scale experimental process (in duplicate) using 20% hamster-adapted 263K scrapie-infected brain homogenate as infective titrated source (10(9) LD50/2 ml), added to each material before being processed and titrated in hamsters. Experiment 1: time/temperature dependency of gelatine autoclaving. Experiment 2: cross-linking and distillation. Experiment 3: final sterilization. Monitoring period: 1 year with daily animal clinical observation. Histology of all brains. SETTING: LCG-RBM laboratories, Italy; strict GLP compliance. MEASUREMENTS AND RESULTS: Heating the gelatine (at conditions lower than those used in production process) was very effective in inactivating the infectivity of TSE agents. Clearance factors were reproducible, dependent upon time and temperature, reaching a total theoretical process clearance in the range of 9.2-13.8 [6.9 + 2.3 (+ 4.6)] log10 LD50. CONCLUSIONS: These experimental results provide further important data confirming the safety of the procedural steps; this complements the safety due to the careful sourcing of the raw material. There is high assurance that there is no significant risk of TSE transmission to humans by the therapeutic administration of polygeline.
     Descriptors: polygeline, gelatine-derived plasma substitutes, bovine derived pharameceutical products, product safety concerns, BSE, transmissible spongiform encephalopathies, manufacturing processes, scrapie, inactivation methods, brain tissue

199. Pen, G.; Cestnik,V. (ed.); Pogacnik, A. BSE -- bovina spongiformna encefalopatija v svetu in stanje v Sloveniji. [BSE -- bovine spongiform encephalopathy in the world and current situation in Slovenia.] 6. Konferenca Slovenske Veterinarske Zveze, 24-25 november 2000, Brdo pri Kranju, Slovenia. Veterinarske-Novice. 2000, 26: Suppl. 1, 51-56; 10 ref. ISSN: 0351-5842. In Slovenian with an English summary.
     Descriptors: BSE, bovine spongiform encephalopathy, prion diseases, cattle diseases, world incidence.

200. Pennington, T. H. The microbiological safety of food. Journal of Medical Microbiology. Aug. 2000. v. 49 (8) p. 677-679
     NAL call no. QR1 J62
     Descriptors: food safety, bacteria, Eubacteria, viruses, prions, pathogens, food born illnesses, public health

201. Perl, S.; Zacharin, I.; Shicaht, N.; Lichawski, D.; Israeli, O.; Said, S. Ben; Orgad, U. BSE survey in Israel and the current status in Western Europe. Israel Journal of Veterinary Medicine. 2000. v. 55 (2) p. 55-58
     Abstract: A report presents the results of a three-year survey (1997-1999) of the incidence of bovine spongiform encephalopathy (BSE) in Israeli cattle. 656 bovine brains were examined, 274 were from the animals presenting with nervous signs (mainly recumbency) and 382 were randomly sampled from abattoirs. None of the bovine brains showed any histopathological lesions of BSE. All 121 brains tested by immunohistochemistry using a specific anti-PrP antibody were found to be negative. The sample size of the animals examined in this study exceeded that required for annual examination to determine that a country be considered free of BSE according to the International Zoosanitation Code of the O.I.E. Based on these results and its compliance with the provisions of the code. Israel is therefore considered free of BSE. The current status of BSE in Western Europe is presented.
     NAL call no. 41.8 R25
     Descriptors: bovine spongiform encephalopathy, incidence in cattle, epidemiology, Israel.

202. Perler, L.; Heim, D.; Geiser, F.; Muller, H. K.; Kihm, U. 10 Jahre BSE in der Schweiz. Der Verlauf einer aussergewohnlichen Krankheit. [Ten years of BSE in Switzerland. The development of an extraordinary disease]. Schweizer Archiv fur Tierheilkunde. Dec 2000. 142(12): 657-664. ISSN: 0036-7281. In German.
     Abstract: The first case of bovine spongiform encephalopathy (BSE) in Switzerland was diagnosed in November 1990. BSE is--in particular considering its eradication--different from many other livestock diseases. Strict disease control measures were taken from the very beginning by the Swiss authorities in order to protect public as well as animal health. In addition, BSE has stimulated enormous media interest and public concern. The occurrence of the born after the ban (BAB) cases, increasing questions about the zoonotic potential of the disease and trade restrictions against Switzerland led to the "BSE-crisis". In 1999, Switzerland internationally took a leading role in the surveillance of BSE by active investigation of targeted risk population.
     NAL call no. 41.8 SCH9
     Descriptors: BSE, history of disease, control measures, zoonotic potential, surveillance program, Switzerland.

203. Peters, J.; Miller, J.M.; Jenny, A.L.; Peterson, T.L.; Carmichael, K.P. Immunohistochemical diagnosis of chronic wasting disease in preclinically affected elk from a captive herd. J Vet Diagn Invest. Lawrence, Kan. : AAVLD. Nov 2000. v. 12 (6) p. 579-582. California; USA. ISSN: 1040-6387
     Abstract: An immunohistochemical (IHC) method was used to test brain tissues from 17 elk in a captive herd in which chronic wasting disease (CWD) had previously occurred. The IHC technique detects the protease-resistant prion protein (PrP-res), which is considered a disease-specific marker for transmissible spongiform encephalopathies (TSE), regardless of the species affected. Of the 17 elk tested, 10 were positive by IHC. Only 2 of these 10 animals had shown clinical signs and histologic lesions of CWD, and an additional animal had histologic lesions only. The most consistently IHC-positive tissue was medulla oblongata, especially the obex. These results show that the PrP-res IHC test on brain tissue, specifically medulla oblongata at the obex, should be considered an essential component of any surveillance study intended to determine the incidence of CWD in captive or free-ranging cervids.
     NAL call no. SF774.J68
     Descriptors: elk, Cervus elaphus canadensis, prion diseases, diagnosis, immunohistochemistry, brain, prion proteins, clinical aspects, lesions, South Dakota, US.

204. The Phillips report on BSE and vCJD. Lancet. 2000 Nov 4; 356(9241): 1535. ISSN: 0140-6736
     NAL call no. 448.8 L22
     Descriptors: BSE, bovine spongiform encephalopathy, Nv Creutzfeldt-Jacob disease, UK government, Spongiform Encephalopathy Advisory Committee, safety of beef products, role of scientific advisory committees in policy, interdepartmental rivalry, Chief Medical Officer, compensation and care for NvCJD patients, scrapie, research initiatives.

205. Phillips, Lord Bridgeman, J.; Ferguson-Smith, M. The BSE Inquiry: the report (Volumes 1-16). London, England: The Stationary Office, 2000.
     Abstract: The inquiry was set up in 1997 to: establish and review the history and emergence and identification of BSE and variant CJD in the UK and of the action taken in response to it up to March 1996; to reach conclusions on the adequacy of that response; and to report the findings to relevant ministries. The resulting report documents in detail the development of two new diseases, one of cattle that devastated the British cattle industry (more than 170 000 animals affected) and another that has caused the deaths of more than 80 people so far. The report identifies several shortcomings in delayed responses and lack of rigour in implementing measures. Poor coordination between health and agricultural departments and bureaucracy impeded prompt responses. The cause of the epidemic was identified as the inclusion of meat and bone meal in ruminant feed. Despite the banning of specified bovine offals in ruminant feed in 1990, cattle born after this date still contracted the disease, probably through cross contamination of feed with feed destined for non-ruminants. The report makes compelling reading for all those who have followed the epidemic. It is available from The Stationary Office (www.thestationeryoffice.com/) in print (pounds sterling 325 for the complete boxed set of 16 volumes (ISBN: 0105569860); or pounds sterling 29.50 for Volume 1: Findings and Conclusions (contains a CD-ROM of the full text of the report; ISBN: 0105569704)), on CD-ROM (pounds sterling 235 which includes the full text of the report on Disc 1. Discs 2-12 include all the witness statements, transcripts, SEAC and ACAF documents and other supporting documents; ISBN: 0105569879), or can be freely accessed on the Internet.
     NAL call no. SF967 S63 B746 2000
     Descriptors: bovine spongiform encephalopathy, Creutzfeldt-Jakob Disease, prion diseases, disease control, epidemiology, cattle, man, UK

206. Piedrahita, J.A. Targeted modification of the domestic animal genome. Theriogenology. New York, N.Y.: Elsevier Science Inc. Jan. 1, 2000. v. 53 (1) p. 105-116. Paper presented at the Annual Conference of the International Embryo Transfer Society, January 9-11, 2000, Maastricht, the Netherlands. Includes references
     Abstract: While the technique of homologous recombination, or gene targeting, has led to the generation of transgenic mice of great value to biomedical research, similar approaches are only being developed in other species. With the exception of recent reports on the generation of gene-targeted sheep, the technology in domestic animals is still in its infancy (45). The development of techniques for generating large animals with deleted or modified genes will result in the generation of animals of great value to society. While the technical difficulties to achieve gene targeting in domestic species are significant, they are not insurmountable. Potential applications in both the bovine and porcine species are described with particular emphasis on the generation of cattle resistant to bovine spongiform encephalopathy (BSE) and pigs that can be of use in xenotransplantation.
     NAL call no. QP251.A1T5
     Descriptors: cattle, pigs, transgenic animals, homologous recombination, nucleotide sequences, DNA sequencing, cell lines, cells, cell culture, senescence, nuclei, chimeras, transplantation, bovine spongiform encephalopathy, prion proteins, genes, targeted mutagenesis, beta-lactoglobulin, literature reviews, stem cells, xenotransplantation, knock-out mutations.

207. Polak, Miroslaw P.; Zmudzinski, Jan F. Diagnosis of transmissible spongiform encephalopathies. Medycyna Weterynaryjna. March 2000. v. 56 (3) p. 143-149
     Abstract: The review describes current diagnostic techniques used for TSEs diagnosis. There is an emphasis on BSE and scrapie in sheep. The clinical signs and their diagnostic reliability are described. Laboratory tests are reviewed. Histopatological examination for BSE is discussed with guidelines for diagnostic criteria on single section examination. Other techniques for PrP detection like: SAF detection, immunohistochemistry and western-blotting have also been presented. The development of novel methods for BSE diagnosis in live animals using cerebrospinal fluid and urine are described.
     NAL call no. 41.8 M463
     Descriptors: TSEs, transmissible spongiform encephalopathies, BSE, bovine spongiform encephalopathies, scrapie, diagnostic methods, histopathology, immunohistochemistry, western blot, SAF detection, PrP.

208. Polak, Miroslaw P.; Zmudzinski, Jan F. The latest information concerning laboratory diagnosis of transmissible spongiform encephalopathies - TSEs. Medycyna Weterynaryjna. 2000. v. 56 (4) p. 211-213
     Abstract: A review describing the European Commission's report concerning the evaluation of diagnostic tests for transmissible spongiform encephalopathy in bovines. Four kits already being marketed or near release were tested. They were examined for the following parameters: specificity, sensitivity and the ability to detect small quantities of PrP as well as reproducibility of the results. Three kits were 100% sensitive and specific, while only one of them detected the smallest quantity of PrP. All the tests are for post-mortem diagnosis of BSE.
     NAL call no. 41.8 M463
     Descriptors: diagnostic tests, PrP, evaluation, transmissible spongiform encephalopathy, cattle, post mortem material.

209. Polak, M. P.; Zmudzinski, J. F.; Rozek, W. New data on the BSE epidemic. [Nowe dane epizootyczne i diagnostyczne na temat BSE.] Magazyn Weterynaryjny. 2000. v. 9 (47) p. 46-47
     Descriptors: BSE, prion diseases, epidemiology, disease prevention, bovine spongiform encephalopathy, cattle, Poland

210. Post, K.; Brown, D. R.; Groschup, M.; Kretzschmar, H. A.; Riesner, D. Neurotoxicity but not infectivity of prion proteins can be induced reversibly in vitro. Archives of virology. Supplementum. 2000. (16): 265-273. ISSN: 0939-1983.
     Abstract: Prion diseases include Creutzfeldt-Jakob disease in humans, scrapie in sheep and bovine spongiform encephalopathy. The hallmark of prion diseases is the accumulation of an abnormal isoform (PrP(Sc)) of the cellular prion protein accompanied by neuronal cell death and astroglial proliferation. To characterize the correlation between PrP secondary and quarternary structure and their biological effects we assayed soluble and aggregated forms of PrP 27-30, the N-terminal truncated form of PrP(Sc), as well as the corresponding recombinant PrP(90-231) for their neurotoxicity and infectivity. PrP was kept soluble in 0.2% SDS and subsequently re-aggregated either by diluting the SDS or by adding acetonitril. The neurotoxicity of the re-aggregated states were comparable to that of prion rods (PrP 27-30) whereas the soluble forms had no neurotoxic effects. The solubilized PrP 27-30 showed no significant infection upon re-aggregation as determined by bioassays in Syrian golden hamsters. The recombinant PrP did not exhibit infectivity in any state.
     NAL call no. QR355 A72
     Descriptors: prion diseases, BSE, scrapie, prion protein isoforms, solubilized PrP27-30, Syrian golden hamster bioassay.

211. Premzl, M.; Bozic, P.; Gamulin, V. PRNP octarepeat allele genotype frequencies among the modern and rare cattle breeds in Croatia. Animal Genetics 2000 vol. 31, no. 6, pp. 408-409. ISSN: 0268-9146
     NAL call no. QP98.A1A5
     Descriptors: prions diseases, BSE, scrapie, PrP, PRNP gene structure, gene comparison in modern breeds of cattle, polymorphism, infected and uninfected comparison.

212. Pretty, J.N.; Brett, C.; Gee, D.; Hine, R.E.; Mason, C.F.; Morison, J.I.L.; Raven, H.; Rayment, M.D.; Bijl, G. van der. An assessment of the total external costs of UK agriculture. Agric Syst. Oxford : Elsevier Science Ltd. Aug 2000. v. 65 (2) p.113-136. ISSN: 0308-521X
     Abstract: This trans-disciplinary study assesses total external environmental and health costs of modern agriculture in the UK. A wide range of datasets have been analyzed to assess cost distribution across sectors. We calculate the annual total external costs of UK agriculture in 1996 to be 2343 pounds m (range for 1990-1996: 1149-3907 pounds m), equivalent to 208 pounds/ha of arable and permanent pasture. Significant costs arise from contamination of drinking water with pesticides (210 pounds m/year), nitrate (16 pounds m), Cryptosporidium (23 pounds m) and phosphate and soil (55 pounds m), from damage to wildlife, habitats, hedgerows and drystone walls (125 pounds m), from emissions of gases (1113 pounds m), from soil erosion and organic carbon losses (106 pounds m) from food poisoning (169 pounds m), and from bovine spongiform encephalopathy (BSE) (607 pounds meter). This study has only estimated those externalities that give rise to financial costs, and so is likely to underestimate the total negative impacts of modern agriculture. These data help to identify policy priorities, particularly over the most efficient way to internalize these external costs into prices. This would imply a redirection of public subsidies towards encouraging those positive externalities under-provided in the market place, combined with a mix of advisory and institutional mechanisms, regulatory and legal measures, and economic instruments to correct negative externalities. Further work examining the marginal costs and benefits of UK agriculture would help to inform future policy development.
     NAL call no. HD1.A3
     Descriptors: externalities, damage, costs, economic impact, agricultural production systems, agricultural policy, pesticides, emissions, soil, biodiversity, nitrates, health risks, environmental impact, drinking water pollution, UK, Scotland, BSE.

213. Priola, S.A.; Raines, A.; Caughey, W.S. Porphyrin and phthalocyanine antiscrapie compounds. Science. Feb. 25, 2000. v. 287 (5457) p. 1503-1506
     NAL call no. 470 SCI2
     Descriptors: transmissible spongiform encephalopathies, TSE, anti-TSE drugs, scrapie, BSE, porphyrins and phthalocyanines, inhibitors of PrP-res, intraperitoneal drug administration, controlled study, mouse model

214. Pulec, J.L. Prions-new infectious agents. Ear, Nose and Throat Journal. 2000. v. 79 (6) p. 419
     Descriptors: prion proteins, transmissible spongiform encephalopathies, BSE, otorhinolaryngology, Creutzfeldt-Jakob disease, scrapie, human tissues

215. Purdey, M. Ecosystems supporting clusters of sporadic TSEs demonstrate excesses of the radical-generating divalent cation manganese and deficiencies of antioxidant co factors Cu, Se, Fe, Zn. Does a foreign cation substitution at prion protein's Cu domain initiate TSE? Medical Hypotheses. 2000. v. 54 (2 p. 278-306)
     Abstract: Analyses of food chains supporting isolated clusters of sporadic TSEs (CWD in N Colorado, scrapie in Iceland, CJD in Slovakia) demonstrate a consistent 2 1/2+ fold greater concentration of the pro-oxidant divalent cation, manganese (Mn), in relation to normal levels recorded in adjoining TSE-free localities. Deficiencies of the antioxidant co factors Cu/Se/Zn/Fe and Mg, P and Na were also consistently recorded in TSE foodchains. Similarities between the clinical/pathological profile of TSEs and Mn delayed psycho-neurotoxicity in miners are cited, and a novel theory generated which suggests that sporadic TSE results from early life dependence of TSE susceptible genotypes on ecosystems characterised by this specific pattern of mineral imbalance. Low Cu/Fe induces an excessive absorption of Mn in ruminants and an increased oxidation of Mn2+ into its pro oxidant species, Mn3+, which accumulates in mitochondria of CNS astrocytes in Mn SOD deficient genotypes. Deficiencies of scavenger co factors Cu/Zn/Se/Fe in the CNS permits Mn3+ initiated chain reactions of auto-oxidant mediated neuronal degeneration to proliferate, which, in turn, up-regulates the expression of the Cu-metalloprotein, prion protein (PrP). Once the rate of PrP turnover and its demand for Cu exceeds the already depleted supply of Cu within the CNS, PrP can no longer bind sufficient Cu to maintain its conformation. Mn3+ substitutes at the vacated Cu domain on PrP, thus priming up a latent capacity for lethal auto-oxidative activity to be carried along with PrP like a 'trojan horse'; where Mn 3+ serves as the integral 'infectious' transmissible component of the misfolded PrP-cation complex. The Mn overactivation of concanavalin A binding to glycoprotein and Mn-initiated autoxidation results in a diverse pathological profile involving receptor capping, aggregation/modification of CNS membrane/cytoskeletal proteins. TSE ensues. The BSE/nvCJD strain entails a 'synthetic' induction of the same CNS mineral disturbance, where 'in utero' exposure to Cu-chelating insecticides/Mn supplements accelerates the onset of a more virulent 'strain' of adolescent TSE.
     Descriptors: food chain analysis, sporatic transmissible spongiform encephalopathies, dietary levels, copper, manganese, selenium, iron and zinc, magnesium, phorphorus, and sodium, prion protein, PrP, mineral imbalance

216. Race, R.; Oldstone, M.; Chesebro, B. Entry versus blockade of brain infection following oral or intraperitoneal scrapie administration: Role of prion protein expression in peripheral nerves and spleen. Journal of Virology. 2000. v. 74 (2) p. 828-833 ISSN 0022-538X
     Abstract: Naturally occurring transmissible spongiform encephalopathy (TSE) diseases such as bovine spongiform encephalopathy in cattle are probably transmitted by oral or other peripheral routes of infection. While prion protein (PrP) is required for susceptibility, the mechanism of spread of infection to the brain is not clear. Two prominent possibilities include hematogenous spread by leukocytes and neural spread by axonal transport. In the present experiments, following oral or intraperitoneal infection of transgenic mice with hamster scrapie strain 263K, hamster PrP expression in peripheral nerves was sufficient for successful infection of the brain, and cells of the spleen were not required either as a site of amplification or as transporters of infectivity. The role of tissue-specific PrP expression of foreign PrP in interference with scrapie infection was also studied in these transgenic mice. Peripheral expression of heterologous PrP completely protected the majority of mice from clinical disease after oral or intraperitoneal scrapie infection. Such extensive protection has not been seen in earlier studies on interference, and these results suggested that gene therapy with mutant PrP may be effective in preventing TSE diseases.
     NAL call no. QR360.J6
     Descriptors: transmissible spongiform encephalopathies, TSE, BSE, bovine spongiform encephalopathy, scrapie, prion protein, PrP, transgenic mouse model, oral or intraperitoneal scrapie infection, gene therapy with mutant PrP, treatment method

217. Race, R. E. The trouble with transmissible degenerative encephalopathy agents. Veterinary Journal. 2000. v. 159 (1) p. 3-4.
     NAL call no. SF601 V484
     Descriptors: transmissible spongiform encephalopathies, prion diseases, bovine spongiform encephalopathy, Creutzfeldt-Jakob Disease, disease transmission.

218. Raymond, G.J.; Bossers, A.; Raymond, L.D.; O'Rourke, K.I.; McHolland, L.E.; Bryant, P.K.-III.; Miller, M.W.; Williams, E.S.; Smits, M.; Caughey, B. Evidence of a molecular barrier limiting susceptibility of humans, cattle and sheep to chronic wasting disease. EMBO J. Oxford, U.K. : Oxford University Press. Sept 1, 2000. v. 19 (17) p. 4425-4430. ISSN: 0261-4189
     Abstract: Chronic wasting disease (CWD) is a transmissible spongiform encephalopathy (TSE) of deer and elk, and little is known about its transmissibility to other species. An important factor controlling interspecies TSE susceptibility is prion protein (PrP) homology between the source and recipient species/genotypes. Furthermore, the efficiency with which the protease-resistant PrP (PrP-res) of one species induces the in vitro conversion of the normal PrP (PrP-sen) of another species to the protease-resistant state correlates with the cross-species transmissibility of TSE agents. Here we show that the CWD-associated PrP-res (PrP(CWD)) of cervids readily induces the conversion of recombinant cervid PrP-sen molecules to the protease-resistant state in accordance with the known transmissibility of CWD between cervids. In contrast, PrP(CWD)-induced conversions of human and bovine PrP-sen were much less efficient, and conversion of ovine PrP-sen was intermediate. These results demonstrate a barrier at the molecular level that should limit the susceptibility of these non-cervid species to CWD.
     NAL call no. QH506.E46
     Descriptors: deer, Cervus elaphus, Odocoileus hemionus, Odocoileus-virginianus, cattle, sheep, man, spongiform encephalopathy, susceptibility, animal proteins, genes, nucleotide sequences, amino acid sequences, recombinant proteins, proteinases, proteolysis, resistance, species differences, disease transmission, chronic wasting disease.

219. Reynaert, Hendrik; Burt, Alastair; Geerts, Albert. Prions in activated hepatic stellate cells: Not a surprise after all. Journal of Hepatology. November, 2000. v. 33 (5) p. 838-841.
     Descriptors: humans, BSE,bovine spongiform encephalopathy, nervous system disease prion disease, Creutzfeldt-Jacob disease, Fatal Familial Insomnia, Gerstmann-Straussler Syndrome, Kuru, scrapie, liver cells.

220. Roberts, D.J.; Lindsay, B. Milk quality and farm assurance issues. Int. J. Dairy Technol. Oxford, U.K. : The Plunkett Foundation. Nov 2000. v. 53 (4) p. 132-134. ISSN: 1364-727X. In the special issue: Dairy Technology 2000, edited by R. Gray. Paper presented at a conference held May 20-23, 2000, Kinsale, Ireland. References.
     NAL call no. SF221.I58
     Descriptors: milk quality, dairy farms, quality controls, somatic cell count, microbial contamination, bacteria, milk fat and protein levels, bovine spongiform encephalopathy, animal welfare.

221. Roels, S.; Charlier, G.; Letellier, C.; Meyer, G.; Schynts, F.; Kerkhofs, P.; Thiry, E.; Vanopdenbosch, E. Natural case of bovine herpesvirus 1 meningoencephalitis in an adult cow. Veterinary Record. 2000. v. 146 (20) p. 586-588, 21 refs. ISSN: 0042-4900.
     NAL call no. 41.8 V641
     Descriptors: cow, case study, encephalitis, Belgian Blue, bovine spongiform encephalopathy, differential diagnosis, meningitis, meningoencephalitis, bovine herpesvirus, bovine herpesvirus 1, Herpesviridae, cattle, Belgium.

222. Rogers, M.; Doyle, D.; Geise, M. Prions: Strategies for control of BSE and scrapie. Irish Journal of Agricultural and Food Research. June 2000. v. 39 (2) p. 229-234
     Abstract: BSE in cattle, scrapie in sheep and Creutzfeldt-Jakob Disease (CJD) in humans are members of a family of fatal neurodegenerative diseases called Transmissible Spongi-form Encephalopathies (TSEs) or Prion diseases. They are a fatal diseases characterized by a long pre-clinical incubation period and a variable length clinical course determined by the variant strain of agent and the host species. The link between BSE in cattle and a variant form of CJD (vCJD) has focused on policies in place to minimize the spread of these agents both in the animal population and transmission to humans. The article discusses strategies for reducing the transmission of these agents and the potential for successful eradication of these animal diseases.
     NAL call no. S539.5 I74
     Descriptors: BSE, bovine spongiform encephalopathy, NvCreutzfeldt-Jakob disease, prion diseases, controls, risk factors of transmission, scrapie, meat products safety.

223. Rowe, G.; Frewer, L.; Sjoberg, L. Newspaper reporting of hazards in the UK and Sweden. Public understanding of science. Jan 2000. 9(1): 59-78. ISSN: 0963-6625.
     Abstract: Public understanding of risks is likely to be informed by the media. We report a cross-national study looking at how newspapers in Sweden and the United Kingdom characterize a variety of risks, focusing on two months around the 10th anniversary of the Chernobyl accident. Approximately four times as many reports about risks were found in Sweden as in the U.K., possibly reflecting a Swedish safety culture. The Bovine Spongiform Encephalopathy (BSE) crisis dominated reporting in both countries, especially in the U.K. The proportion and pattern of reports on Chernobyl was similar across countries. However, in Sweden, there was an increase in reports about other nuclear hazards after the anniversary, suggesting that generalization of media concern may have occurred. Generally, BSE was discussed using a greater number of characterizations in the U.K., while Chernobyl was reported using more characterizations in Sweden. Reports about hazards tended to be alarmist rather than reassuring, and rarely used statistics to express degrees of risk.
     Descriptors: media, newspapers, reporting on risks, BSE, country comparison, Sweden, UK.

224. Ryder, S.J.; Hawkins, S.A.C.; Dawson, M.; Wells, G.A.H. The neuropathology of experimental bovine spongiform encephalopathy in the pig. Journal of Comparative Pathology. London: W.B. Saunders Company Ltd. Feb./April 2000. v. 122 (2/3) p. 131-143. Includes references
     Abstract: In an experimental study of the transmissibility of BSE to the pig, seven of 10 pigs, infected at 1-2 weeks of age by multiple-route parenteral inoculation with a homogenate of bovine brain from natural BSE cases developed lesions typical of spongiform encephalopathy. The lesions consisted principally of severe neuropil vacuolation affecting most areas of the brain, but mainly the forebrain. In addition, some vacuolar change was identified in the rostral colliculi and hypothalamic areas of normal control pigs. PrP accumulations were detected immunocytochemically in the brains of BSE-infected animals. PrP accumulation was sparse in many areas and its density was not obviously related to the degree of vacuolation. The patterns of PrP immunolabelling in control pigs differed strikingly from those in the infected animals.
     NAL call no. 41.8 J82
     Descriptors: pigs, bovine spongiform encephalopathy, experimental infections, pathology, histopathology, disease transmission, susceptibility, lesions, brain, vacuoles, prion proteins, immunocytochemistry.

225. Salzberger, B.; Franzen, C.; Fatkenheuer, G. Update in infectious diseases. Part I: Epidemiology. [Update infektiologie. Teil I: Epidemiologie.] Medizinische Klinik. June 15, 2000. v. 95 (6) p. 314-320
     Abstract: A number of infectious agents has been newly detected in the last 10 years. Climatic changes and migration have been the most important factors in the emergence of new and old infections. Additionally, new methods for the detection of DNA and RNA have played an important role in the detection of agents difficult to culture. Relevant new bacterial pathogens are Bartonella henselae (cat scratch disease, bacillary angiomatosis), Tropheryma whippeli (Whipple's disease) and new Rickettsiae. Newly detected viral pathogens include Sin-nombre virus (pulmonary Hanta virus syndrome), Nipah- and Hendra virus and avian influenza. Bovine spongiform encephalopathy has been transmitted to humans causing the newly described syndrome of variant Creuzfeldt-Jakob disease. The extent of this new epidemic is not yet clear. These trends from the last years clearly indicate, that further new infections and infectious agents will be detected in the future.
     Descriptors: Bartonella henselae, Tropheryma whippeli, Rickettsiae, Sin-nombre virus, Nipah- and Hendra virus and avian influenza, BSE, bovine spongiform encephalopathy, Creuzfeldt-Jakob disease, emerging zoonotic diseases.

226. Schlapfer J.; Stahlberger-Saitbekova N.; Kuffer J.; Dolf, G. Genetic mapping of the prion protein gene (PRNP) on bovine chromosome 13. Journal of Animal Breeding and Genetics. June 2000. v. 117 (3) p. 211-216
     Abstract: The bovine prion protein gene (PRNP) potentially plays a key role in the development of bovine spongiforme encephalopathy (BSE). In species other than cattle, expression of BSE is clearly due to polymorphisms in this gene. PRNP has previously been assigned to the bovine chromosome 13 (BTA13). The present study is an attempt to embed PRNP into a grid of published marker maps. A genetic mapping panel consisting of 266 animals has been genotyped with 19 microsatellites and a polymerase chain reaction-amplified polymorphism within the PRNP coding region. The linear locus order and the relative distances of these loci are presented. Our linkage map spans 111.6 cM of BTA13. The results suggest PRNP to be located telomeric of the microsatellite BMS1580 and centromeric of BM9248 with a log-likelihood of gtoreq 2. Our findings further characterize the vicinity of PRNP on BTA13.
     NAL call no. 442.8 Z35
     Descriptors: molecular genetics, chromosome 13 [BTA13], PRNP location, prion protein, genetic mapping, cattle, bovine spongiform encephalopathy.

227. Schulz-Schaeffer, W. J.; Fatzer, R.; Vandevelde, M.; Kretzschmar, H. A. Detection of PrP(Sc) in subclinical BSE with the paraffin-embedded tissue (PET) blot. Archives of virology. Supplementum. 2000. (16): 173-180. ISSN: 0939-1983.
     Abstract: The appearance of a new variant of CJD (vCJD) in young patients has caused considerable public concern and there is evidence that this novel disease is caused by the same agent as BSE. BSE is a prion disease that became epidemic in the UK, with a peak incidence in January 1993. New test systems should aim to identify BSE-infected cattle early in the incubation period. We compared the established histological and immunohistochemical methods and the Western blot method used by Prionics with the PET blot method that detects prion PrP(Sc) deposits in formalin-fixed and paraffin-embedded tissue. Investigating the obex region with the PET blot, all BSE cases were detectable and no false positive cases occurred. From the Swiss culling program, five clinically healthy cattle out of 1761 were identified as incubating BSE. With the PET blot method four of them showed the same PrP(Sc) deposition pattern that was seen in clinical BSE, though less conspicuous. In one of the five cases, PrP(Sc) was restricted to two brain stem nuclei, a pattern that was reported to be the first manifestation of PrP(Sc) deposits in the brain after peripheral infection and one that occurs after half of the incubation time. In this case, histology and Western blot were negative.
     NAL call no. QR355 A72
     Descriptors: NvCreutzfeldt-Jakob Disease, cattle, diagnostic test, Prionics western blot, comparison of methods, PET blot method, screening of culled animals, Switzerland.

228. Schulz-Schaeffer, W. J.; Tschoke, S.; Kranefuss, N.; Drose, W.; Hause-Reitner, D.; Giese, A.; Groschup, M. H.; Kretzschmar, H. A. The paraffin-embedded tissue blot detects PrP(Sc) early in the incubation time in prion diseases. American journal of pathology. Jan 2000. 156(1): 51-56. ISSN: 0002-9440.
     Abstract: With the appearance of bovine spongiform encephalopathy (BSE) and a new variant of Creutzfeldt-Jakob disease (nvCJD) that seems to be caused by BSE, there is an increased need for improvement of diagnostic techniques and recognition of all variants of prion diseases in humans and animals. Publications on the immunohistochemical identification of PrP(Sc) in the tonsils and appendix in the incubation period of nvCJD indicate that new and more sensitive techniques for the detection of PrP(Sc) in various tissues may be a valuable tool for early diagnosis in prion diseases. We developed a new and sensitive technique to detect PrP(Sc) in formalin-fixed and paraffin-embedded tissue, the paraffin-embedded tissue blot (PET blot), and reinvestigated archival brain material from CJD as well as BSE and scrapie. In addition, C57/Bl6 mice experimentally infected with the ME7 strain were investigated sequentially during the incubation time to compare this new technique with conventional methodologies. The PET blot detects PrP(Sc) in idiopathic (sporadic) and acquired prion diseases, even in cases with equivocal or negative immunohistochemistry, and is more sensitive than the conventional Western blot and histoblot techniques. The PET blot makes possible the detection of PrP(Sc) during the incubation period long before the onset of clinical disease and in prion disease variants with very low levels of PrP(Sc). In mice experimentally infected with the ME7 strain, the PET blot detects PrP(Sc) in the brain 30 days after intracerebral inoculation-145 days before the onset of clinical signs. Its anatomical resolution is superior to that of the histoblot technique. It may therefore be of particular interest in biopsy diagnosis. Thus it complements other tissue-based techniques for the diagnosis of prion diseases in humans and animals.
     NAL call no. 448.8 AM39
     Descriptors: diagnostic techniques, prion proteins, archival brain tissue, CJD, scrapie, BSE, experimental infection, C57/B15 mouse model, incubation period, prion disease.

229. Science and politics - A broken union. Nature Medicine. 2000. v. 6 (12) p. 1295.
     Descriptors: biomedicine, politics, medical research, BSE, bovine spongiform encephalopathy, embryo cell, stem cell transplantation, zoonosis, Creutzfeldt-Jakob disease, United Kingdom, financial management, policy.

230. Scott, M.R.; Supattapone, S.; Nguyen, H. O.; DeArmond, S. J.; Prusiner, S. B. Transgenic models of prion disease. Archives of virology. Supplementum. 2000. (16): 113-124. ISSN: 0939-1983.
     Abstract: There is growing concern that bovine spongiform encephalopathy (BSE) may have passed from cattle to humans, resulting in approximately 70 cases of an atypical, variant CJD (vCJD) in teenagers and young adults. We report here that transgenic (Tg) mice expressing full-length bovine (Bo) PrP serially propagate BSE prions and that there is no species barrier for transmission from cattle to Tg(BoPrP) mice. Surprisingly, these same mice were also highly susceptible to vCJD and natural sheep scrapie. The incubation times (approximately 250 d), neuropathology, and PrP(Sc) isoforms in Tg(BoPrP) mice inoculated with vCJD and BSE brain extracts were indistinguishable and differed dramatically from those seen in these mice injected with natural scrapie. In efforts to identify PrP sequences required for prion formation, we found that a redacted prion protein of only 106 amino acids (PrP106) containing two large deletions supported prion propagation. In Tg(PrP106) mice, an artificial transmission barrier for the passage of full-length mouse prions was diminished by the coexpression of full-length wt MoPrP(C), suggesting that wt MoPrP acts in trans to accelerate the replication of "miniprions" containing PrP(Sc)106. Following a single passage (approximately 300 d) in Tg(PrP106) mice, the miniprions efficiently transmitted disease to Tg(PrP106) mice after only approximately 66 days. Our findings with Tg(BoPrP) mice provide compelling evidence that prions from cattle with BSE have infected humans and caused fatal neurodegeneration, the unique features of miniprions offer new insights into the mechanism of prion replication, and the trans-acting effects of full-length PrP coexpression suggest a new approach to the development of even more efficient animal models for prion diseases.
     NAL call no. QR355 A72
     Descriptors: transgenic mice, disease model, human and animal prion diseases, NvCreutzfelt-Jakob Disease, prion strains, species barriers, prion propagation.

231. Separovic, S.; Brstilo, M.; Labrovic, A.; Sosic, B.B. Goveda spongiformna encefalopatija: od problema utvrdivanja stanja zdravlja nacionalnog stada do najeksponiranijeg problema javnog zdravstva i promisljenog kreiranja buducih propisa. [Bovine spongiform encephalopathy: establishing the national herd health status and the most prominent problems of public health and prudence in drafting future regulations.] Drugi Hrvatski Veterinarski Kongres, Cavtat, 10-13 Listopada 2000. Second Croatian Veterinary Congress: Proceedings, Cavtat, Croatia, 10-13-October-2000. 2000, 343-356; 18 ref. Pub. Hrvatsko Veterinarsko Komora (Croatian Veterinary Association); Zagreb; Croatia ISBN: 953-96576-8-7. Conference paper in Croatian with an English summary.
     Descriptors: BSE, bovine spongiform encephalopathy, public health, legislation, law, livestock.

232. Sharma, R.K.; Arun, Kumar; Thapliyal, D.C.; Kumar, A Bovine spongiform encephalopathy - a review. Indian Journal of Comparative Microbiology, Immunology and Infectious Diseases. 2000, 21: 2, 94-97; 34 ref. ISSN: 0970-9320
     Descriptors: BSE, etiology, pathology, BSE, bovine spongiform encephalopathy; clinical aspects; disease prevention and control, zoonotic disease transmission, prion diseases, humans, pathogenesis.

233. Shaw, A. Public understanding of food risks: what do the experts say? Food Science and Technology Today. 2000, 14: 3, 140-143. ISSN: 0950-9623
     NAL call no. TX341.F664
     Descriptors: food safety concerns, public health, consumer attitudes, microbial contamination, genetic-engineered food risk factors, surveys, research, BSE, bovine spongiform encephalopathy

234. Shickle, D. "On a supposed right to lie [to the public] from benevolent motives": communicating health risks to the public. Medicine, health care, and philosophy. 2000. 3(3): 241-249. ISSN: 1386-7423.
     Abstract: There are three main categories of rationale for withholding information or telling lies: if overwhelming harm can only be averted through deceit; complete triviality such that it is irrelevant whether the truth is told; a duty to protect the interests of others. Public health authorities are frequently having to form judgements about the public interest, whether to release information or issue warnings. In June 1992, routine surveillance detected patulin levels (a known carcinogen) in samples of apple juice exceeding safety threshold. Remedial actions were promptly taken and it was planned to subsequently publish the information in the routine way. However, the media portrayed the handling of the problem as a conspiracy and there was a short term reduction in juice sales. In October 1995, the UK Committee on Safety of Medicines issued a warning about certain brands of the contraceptive pill, based on the interim results of three unpublished studies. The increased risk of thromboembolism was small, but the resulting scare led to an increase in unwanted pregnancies. The handling of the B.S.E. crisis in the U.K. also led to accusations of incompetence or conspiracy. Public health authorities have to handle uncertainty and frequently have to form judgements for public safety on the basis of evidence of poor quantity and quality. Their task is not helped by the sometimes conflicting agenda of scientists and media. The public also have differing perceptions and interpretations of risk. The series of scares and crises are having a detrimental effect on public confidence in public health authorities.
     Descriptors: public health communication, BSE, UK, evidence based decision making, public perceptions.

235. Shirley, R.B.; Parsons, C.M. Effect of pressure processing on amino acid digestibility of meat and bone meal for poultry. Poultry Sci. Savoy, IL : Poultry Science Association, Inc. Dec 2000. v. 79 (12) p. 1775-1781. ISSN: 0032-5791
     Abstract: In the future, it may become desirable or required to process meat and bone meal (MBM) under pressure to reduce human health concerns associated with bovine spongiform encephalopathy (BSE). Therefore, three experiments evaluated the effects of different processing pressures on the digestibility of amino acids (AA) in MBM when the pressure processing was done after typical rendering (Experiments 1 and 2) or during the initial rendering process of raw materials (Experiment 3). Processing pressures varied from 0 to 60 psi in experimental or commercial feather meal cookers. Increasing pressure during processing reduced MBM Cys concentrations in Experiments 1 and 2. True digestibilities of most AA were significantly decreased by increasing pressures in Experiments 1 and 2, and reductions were generally largest for Cys and Lys, particularly Cys, and increased with severity as pressure increased. For example, in Experiment 1, Cys digestibility decreased from 65 to 50 to 15%, and Lys digestibility decreased from 76 to 68 to 41% as the MBM was processed at 0, 30, and 60 psi, respectively, for 20 min. When the pressure processing occurred during the initial rendering of the MBM raw material (Experiment 3), a significant reduction in digestibility of most AA was observed only at 60 psi, and the decrease was much less than that observed in Experiments 1 and 2. Our results indicate that pressure processing of MBM decreases the digestibility of AA for poultry. Thus, pressure processing of MBM to reduce potential BSE infectivity will likely decrease the nutritional value of the MBM.
     NAL call no. 47.8 Am33P
     Descriptors: meat and bone meal, proximate analysis, heat treatment, pressure, rendering, amino acids digestibility, cysteine, lysine, nutritive value, bovine spongiform encephalopathy, disease transmission.

236. Silvestri, G.; Baldassarre, F. Prion diseases: A typical Kuhnian abnormality in a molecular paradigm. Medical Hypotheses. 2000. v. 54 (1) p. 69-71
     Descriptors: spongiform encephalopathies, scrapie, BSE, kuru, Creutzfeldt-Jacob disease, fatal familial insomnia, prions, PrP, molecular structure, protein only model of disease

237. Simon, J. L.; Gillot, D.; Matthes, W.; Sanftleben, P.; Golisch, D. The French beef industry: how can consumer expectations be met? [Franzosische Rinderzucht: Wie konnen die Erwartungen der Verbraucher erfullt werden?] Effiziente tierische Leistung in Verbindung mit einer nachhaltingen Produktion. 3. Wilhelm-Stahl-Symposium am 16. Und 17. Mai 2000 in Rostock. Archiv fur Tierzucht. 2000. v. 43 (Special) p. 91-97
     Abstract: Measures taken to regain the confidence of French consumers since the emergence of BSE are discussed.
     NAL call no. 49 AR23
     Descriptors: bovine spongiform encephalopathy, consumer confidence, beef meat products, meat hygiene, labelling, meat quality, quality controls, certification, France.

238. Sivakumaran, M.; Holada, K.; Simak, J.; Vostal, J.G. Transmission of BSE by blood transfusion [10] (multiple letters). The Lancet. Nov. 18, 2000. v. 356 (9243) p. 1771-1772 ISSN: 0099-5355
     NAL call no. 448.8 L22
     Descriptors: BSE, blood transfusion, bovine spongiform encephalopathy, disease transmission risks, immunohistochemistry, blood sampling, letters.

239. Sostaric, B.; Lipej, Z.; Lojkic, M.; Brstilo, M.; Separovic, S. Dijagnostika animalnih spongiformnih encefalopatija u Hrvatskoj. [Diagnostics of animal spongiform encephalopathies in Croatia.] Drugi Hrvatski Veterinarski Kongres, Cavtat, 10-13 Listopada 2000. Second Croatian Veterinary Congress: Proceedings, Cavtat, Croatia,-10-13-October-2000. 2000, 333-341; 2 ref. Pub: Hrvatsko Veterinarsko Komora, Zagreb, Croatia. ISBN: 953-96576-8-7. Conference paper in Croatian with an English summary.
     Descriptors: BSE diagnosis, diagnostic techniques, prion diseases, disease prevention and control.

240. Stevenson, M.A.; Wilesmith, J.W.; Ryan, J.B.M.; Morris, R.S.; Lawson, A.B.; Pfeiffer, D.U.; Lin, D. Descriptive spatial analysis of the epidemic of bovine spongiform encephalopathy in Great Britain to June 1997. The Veterinary record: journal of the British Veterinary Association. London: The British Veterinary Association. Sep. 30, 2000. v. 147 (14) p. 379-384. Includes references
     Abstract: This was a spatial analysis of the epidemic of bovine spongiform encephalopathy (BSE) in Great Britain, based on agricultural census data collected between 1986 and 1996 and BSE case data collected up to June 1997. Kernel smoothing techniques were used to plot the distribution of BSE-positive cattle holdings per 100 holdings per square kilometre and the distribution of confirmed BSE cases per 100 head of cattle per square kilometre. In the early stages of the epidemic reported BSE cases were scattered widely throughout Great Britain, with no clearly identifiable focus. By June 1997, a statistically significant cluster of BSE-positive holdings was identifiable in the eastern part of the South west region of England. During the epidemic the highest densities of confirmed BSE cases per 100 cattle per square kilometre occurred in the greater part of the South west region of England and within Dyfed in the south west of Wales. In Wales, a small number of holdings experienced large numbers of confirmed BSE cases. In the South west region of England a large number of holdings experienced small numbers of confirmed cases. By June 1997, the distribution of BSE-positive holdings across Great Britain was largely determined by factors that influenced the amount of recycled infectious material they were exposed to.
     NAL call no. 41.8 V641
     Descriptors: cattle, bovine spongiform encephalopathy, epidemics, disease distribution, spatial distribution, Great Britain.

241. Stevenson, M.A.; Wilesmith, J.W.; Ryan, J.B.M.; Morris, R.S.; Lockhart, J.W.; Lin, D.; Jackson, R. Temporal aspects of the epidemic of bovine spongiform encephalopathy in Great Britain: individual animal-associated risk factors for the disease. The Veterinary record: journal of the British Veterinary Association. London: The British Veterinary Association. Sep. 23, 2000. v. 147 (13) p. 349-354. Includes references.
     Abstract: The objectives of this study were first to determine the cumulative incidence of bovine spongiform encephalopathy (BSE) in the British cattle population from July 1986 to June 1997, secondly, to identify individual animal-associated risk factors that influenced the age of onset of clinical signs in confirmed BSE cases, and, thirdly, to assess the effectiveness of the measures introduced to control BSE during the epidemic. The analyses were based on the population of British cattle at risk, derived from agricultural census data collected between 1986 and 1996, and BSE case data collected up to June 30, 1997. The unit of interest was individual adult cattle recorded on annual agricultural censuses between June 1986 and June 1996. Univariate and multivariate survival analysis techniques were used to characterise the age of onset of clinical signs. In total 167,366 cases of BSE were diagnosed in Great Britain up to June 30, 1997. The cumulative incidence of BSE between July 1986 and June 1997 was 1.10 (95 per cent confidence interval [CI] 1.09 to 1.10) cases per 100 adult cattle at risk. Cattle from the South east, South west and Eastern regions of England had 4.26 to 5.96 (95 per cent CI 4.15 to 6.14) times as great a monthly hazard of being confirmed with BSE as cattle from Scotland. Compared with cattle born before June 1985, those born between July 1987 and June 1988 had 22.5 (95 per cent CI 22.1 to 22.8) times the monthly hazard of being confirmed with BSE, whereas those born in the 12 months after July 1988 had only 7.39 (95 per cent CI 7.24 to 7.54) times the monthly hazard of being confirmed with BSE. This reduction in hazard was directly attributable to the ban on the use of ruminant protein as a feed instituted in July 1988. Successive cohorts from 1989 to 1991 experienced further reductions in the hazard of experiencing BSE. The additional decrease in hazard observed for the 1990 cohort may be attributed to the effect of the Specified Bovine Offal ban instituted in September 1990.
     NAL call no. 41.8 V641
     Descriptors: cattle, bovine spongiform encephalopathy, risk factors, age, clinical aspects, disease control, efficacy, epidemics, disease prevalence, Great Britain.

242. Suarez-Fernandez, G. Presente y futuro de la epidemiologia infecciosa. [Present and future of infectious epidemiology]. Anales de la Real Academia Nacional de Medicina. 2000. 117(1): 187-201; discussion 201-206. ISSN: 0034-0634. In Spanish.
     Abstract: A revision is made on the important biological phenomenon of microbial emergency, based on model infection diseases in which the author can give the own experimental results and experience. The concept of microbial emergency has shocked the traditional infectious epidemiology since 1983 when the HIV virus was discovered and the informatic network reached an optimum efficiency. In despite of the global risk of contagion it is necessary to consider the proximity effect in a space-time model as the main cause of infectious diseases diffusion.
     Descriptors: modelling infectious disease, eipdemiology, microbial diseases.

243. Sundlof, S.F. Protecting consumers by safeguarding animal health. American Family Physician. Aug. 1, 2000. v. 62 (3) p. 659-660
     Descriptors: consumers, public health service, veterinary medicine, health care delivery, BSE, bovine spongiform encephalopathy, US Food and Drug Administration, food irradiation.

244. Sweeney, T.; Kuczius, T.; McElroy, M.; Parada, M. G.; Groschup, M. H. Molecular analysis of Irish sheep scrapie cases. Journal of General Virology. 2000. v. 81 (6) p. 1621-1627, 33 refs. ISSN: 0022-538X
     Abstract: Different strains of transmissible spongiform encephalopathies in humans and rodent models are associated with the accumulation of PrP(Sc) of distinct molecular characteristics. These characteristics include glycosylation profiles, fragment sizes and long-term resistance of PrP(Sc) to proteinase K. The first objective of this study was to determine the applicability of these criteria to characterize and differentiate sheep scrapie PrP(Sc) and bovine spongiform encephalopathy (BSE) PrP(Sc). PrP(Sc) in sheep scrapie samples from Ireland had clearly distinct molecular characteristics to PrP(Sc) in cattle BSE samples using a monoclonal antibody (MAb P4) directed to position 89-104 of ovine PrP using either brain homogenates or semi-purified scrapie-associated fibrils. Similar glycoprofiles were found when analysing scrapie PrP(Sc) in six different CNS regions (thoracic spinal cord, thalamus, basal ganglia, mediobasal hypothalamus, medulla oblongata and cortex). While the long-term resistance results using a different monoclonal antibody (raised to ruminant PrP positions 145-163; MAb L42) were similar to the results obtained with MAb P4, different glycotyping results were obtained. Given the variation in glycosylation patterns using different antibodies, we conclude that standardization of methodology and antibodies is crucial to the applicability of molecular analysis of ruminant BSE and scrapie samples.
     NAL call no. QR360.J6
     Descriptors: transmissible spongiform encephalopathies, glycosylation profiles, PrPSc, prion protein, scrapie, sheep, comparison study of prion strains, monoclonal antibodies, brain and spinal cord, molecular analysis.

245. Tavernier, J. de; de Tavernier, J.; Dagevos, H. (ed.); Frouws, J. Consumentenzorgen en risicoperceptie. [Consumer concerns and perception of risk.] A confidence issue: consumer concerns and the agrosector, TSL study day, 20 April 2000, Rijswijk, Netherlands. Tijdschrift voor Sociaalwetenschappelijk Onderzoek van de Landbouw. 2000, 15: 2-3, 98-101; 5 ref. ISSN: 0921-481X. In Dutch.
     Descriptors: BSE, bovine spongiform encephalopathy, cattle diseases, consumers concerns, risk perception and assessment, food policy, food safety, prion diseases.

246. Taylor, D.M. Inactivation of transmissible degenerative encephalopathy agents: A review. Veterinary Journal. Jan. 2000. v. 159 (1) p. 10-17
     Abstract: The agents causing transmissible degenerative encephalopathies, such as bovine spongiform encephalopathy, scrapie, and Creutzfeldt-Jakob Disease (CJD), are relatively resistant to inactivation by standard decontamination procedures. The only methods appearing to be completely effective under worst-case conditions are strong sodium hypochlorite solutions or hot solutions of sodium hydroxide. Other procedures that result in significant degrees of inactivation are described. The infectivity levels in histologically-fixed tissue can be reduced substantially by treatment with concentrated formic acid without adversely affecting the microscopic quality of the tissue.
     NAL call no. SF601 V484
     Descriptors: BSE, bovine spongiform encephalopathy, BSE, scrapie, Creutzfeldt-Jakob Disease CJD, decontamination, sodium hypochlorite, sodium hydroxide, formic acid, literature review

247. Taylor, D. M.; Fernie, K.; Reichl, H. E.; Somerville, R. A. Infectivity in the blood of mice with a BSE-derived agent. Journal of Hospital Infection. Sep., 2000. 46 (1) p. 78-79
     Descriptors: BSE, bovine spongiform encephalopathy, mouse infectivity model, blood, prion transfer.

248. Tranulis, M. A.; Espenes, A.; Gunnes, G.; Heggebo, R. From slow virus to prion - the molecular pathology of prion diseases. [Fra slow virus til prion. Prionsykdommenes molekylaere patologi.] Norsk Veterinaertidsskrift. 2000. v. 112 (5) p. 330-341, 81 refs. Special issue of 13 papers on a variety of topics including scrapie, bovine spongiform encephalopathy, and Creutzfeldt-Jakob Disease prion diseases, epidemiology of scrapie in Norway and Iceland, control programs, incidence of prion disease in Norway, BSE in Europe and Norwegian surveillance programs.
     NAL call no. 41.8 N81
     Descriptors: Creutzfeldt-Jakob Disease, bovine spongiform encephalopathy, scrapie, prion diseases, transmissible spongiform encephalopathy, pathogenesis, reviews.

249. Turner, Marc L.; Ludlam, Christopher A. Variant Creutzfeldt-Jakob Disease. Transfusion Medicine Reviews. July 2000. v. 14 (3) p. 216-222.
     Descriptors: human prion disease, genetic variants, transmissible spongiform encephalopathy, disease transmission, risk factors, NvCreutzfeldt-Jakob Disease.

250. Ulvund, M. J.; Kvamsdal, H.; Hagen, G. Bovine spongiform encephalopathy (BSE) in Great Britain. [Bovin spongiform encefalopati (BSE) i Storbritannia. Epidemiologiske forhold og ringvirkninger i Europa.] Norsk Veterinaertidsskrift. 2000. v. 112 (5) p. 386-395, 51 refs. Special issue of 13 papers on a variety of topics including scrapie, bovine spongiform encephalopathy, and Creutzfeldt-Jakob Disease prion diseases, epidemiology of scrapie in Norway and Iceland, control programs, incidence of prion disease in Norway, BSE in Europe and Norwegian surveillance programs.
     NAL call no. 41.8 N81
     Descriptors: bovine spongiform encephalopathy, BSE, prion diseases, epidemiology, cattle, UK.

251. United States. Department of Agriculture, Foreign Agricultural Service. BSE in Europe: the impact on U.S. and world soybean and soybean meal demand. Oilseeds: World Markets and Trade. 2000, No. FOP 12-00, 5-6.
     NAL call no. aHD9490 A2U5
     Descriptors: bovine spongiform encephalopathy, disease control, feed grains demand, feeding, feeds, imports, soybean oilmeal, alternative to animal proteins in foods, meat and bone meal.

252. United States. General Accounting Office. Food safety controls can be strengthened to reduce the risk of disease linked to unsafe animal feed: report to the Honorable Richard J. Durbin, United States Senator /United States General Accounting Office. Washington, D.C.: GAO, [Sep. 2000]. 27 p.
     NAL call no. TX531.U55 2000
     Descriptors: food adulteration and inspection, feeds, bovine spongiform encephalopathy, food safety, US General Accounting Office report.

253. United States. Public Health Service. Public health service recommendations for the use of vaccines manufactured with bovine-derived materials. MMWR Morbidity and Mortality Weekly Report. 2000 Dec 22; 49(50): 1137-8 ISSN: 0149-2195
     Abstract: The Center for Biologics Evaluation and Research (CBER), U.S. Food and Drug Administration (FDA) learned earlier this year that some vaccines were manufactured with bovine-derived materials obtained from countries in which bovine spongiform encephalopathy (BSE) or a substantial risk for BSE exists. A list of these countries is published by the U.S. Department of Agriculture (USDA). This information was of concern because cases of variant Creutzfeldt-Jakob disease (vCJD) have been attributed to, among other possibilities, eating beef products from cattle infected with the agent of BSE. No evidence exists that cases of vCJD are related to the use of vaccines, and no cases of vCJD have been reported in the United States.
     NAL call no. RA407.3 M56
     Descriptors: U.S. Center for Biologics Evaluation and Research, CBER, Food and Drug Administration, FDA, vaccine safety, possible contamination of bovine derived materials, imported products, Nv Creutzfeldt-Jakob Disease.

254. Van Everbroeck, B.; Pals, P.; Dziedzic, T.; Dom, R.; Godfraind, C.; Sciot, R.; Brucher, J.M.; Martin, J.J.; Cras, P. Retrospective study of Creutzfeldt-Jakob Disease in Belgium: Neuropathological findings. Acta Neuropathologica. 2000. v. 99 (4) p. 358-364
     Abstract: Creutzfeldt-Jakob disease (CJD) is a spongiform encephalopathy that affects about 1 in 10(6) inhabitants in most countries. Recently, a new variant of CJD has been linked to the epidemic of bovine spongiform encephalopathy. Therefore, vigilance concerning the disease's incidence has been increased. We conducted a comprehensive, nation-wide and retrospective study. In 79 Belgian autopsies, we found the characteristic triad of spongiosis, neuronal loss and reactive gliosis. The occipital cortex was most affected, while the cerebellum was mostly spared. Immunohistochemistry was performed using hydrated autoclave pretreatment and several monoclonal antibodies directed against the prion protein. We identified prion-immunoreactive patterns and locations reflecting the important heterogeneity, independently of the antibody that was used. Granular prion immunoreactivity was observed in astrocytes. We studied the regional intensity of the prion immunostaining and determined that the frontal cortex with 95% positive immunoreactivity was best suited for a biopsy. We studied the disease duration in sporadic CJD patients who showed neuropathological lesions of other neurodegenerative disorders (such as Alzheimer's disease). The study shapes the framework in which a prospective neuropathological registry will be able to function.
     Descriptors: NvCreutzfeldt-Jakob Disease, transmissible spongiform encephalopathies, BSE, bovine spongiform encephalopathy, immunohistochemistry, prion protein, PrP, human tissue analysis, prospective neuropathological registry, Belgium

255. Van Keulen, L. J. M.; Langeveld, J. P. M.; Garssen, G. J.; Jacobs, J. G.; Schreuder, B. E. C.; Smits, M. A. Diagnosis of bovine spongiform encephalopathy: A review. Veterinary Quarterly. October, 2000. v. 22 (4) p. 197-200.
     Abstract: Cows affected with bovine spongiform encephalopathy (BSE) display chronic neurological signs consisting of behavioral changes, abnormalities of posture and movement, and/or hyperaesthesia. Currently, there is no diagnositic test for BSE in the live animal. In this article, we describe the post-mortem diagnostic examination of brains from BSE-suspected cattle as currently performed at ID-Lelystad. The routine laboratory diagnosis of BSE consists of histopathological examination of the brain and detection of the modified prion protein, PrPBSE, in brain tissue. These tests take several days to complete. Recently, at ID-Lelystad a new post-mortem test has been developed that enables screening of larger volumes of brain samples for PrPBSE within 1 day. This BSE test is especially suited for use in slaughter houses. A preliminary validation study has shown that both sensitivity and specificity are 100% compared to the gold diagnostic standard of histopathology.
     NAL call no. SF601 V46
     Descriptors: cattle brains, diagnostic tests, BSE, bovine spongiform encephalopathy, prions, slaughter house applications.

256. Van Knapen, F.; Zaaijer, H.L. Bovine spongiform encephalopathy and the safety of food [1]. [Boviene spongiforme encefalopathie en de veiligheid van voedsel.] Nederlands Tijdschrift voor Geneeskunde. Aug. 26, 2000. v. 144 (35) p. 1703-1704
     Descriptors: BSE, bovine spongiform encephalopathy, food safety, risk factors, European Union, Creutzfeldt-Jakob disease

257. Vanopdenbosch, E.; Roels, S. BSE en variant CJZ: nieuwe inzichten en achtergrondinformatie. [BSE and variant CJD: new insights and background information.] Vlaams Diergeneeskundig Tijdschrift. 2000, 69: 6, 371-376. ISSN: 0303-9021. In Dutch.
     NAL call no. 41.8 V84
     Descriptors: BSE, bovine spongiform encephalopathy, cattle, prion diseases, NvCreutzfeldt-Jakob disease.

258. Verbeke, W.; Ward, R.W.; Viaene, J. Probit analysis of fresh meat consumption in Belgium: exploring BSE and television communication impact. Agribusiness: an international journal. New York: John Wiley & Sons, Inc. Spring 2000. v. 16 (2) p. 215-234. Includes references
     Abstract: This article focuses on factors influencing consumer decision making toward fresh meat consumption in Belgium. Discrete choice models are specified for explaining consumer decisions to decrease fresh meat consumption since the BSE-crisis and toward to the future. Demographic consumer characteristics, consumption frequency and attention to television coverage are included as explanatory variables in the models. A major focus is the impact of television, which has carried several negative reports about meat safety during recent years. Television coverage is found to have a highly negative impact on decision making toward fresh red meat consumption. Model estimation and computation of predicted probabilities reveal that the likelihood of cutting fresh meat consumption increases with greater attention given to television messages, as well as with the presence of young children in the household and with increasing age of the consumer. Interaction between attention to television and age reveals that younger people's decisions are more susceptive to media coverage. Heavy meat consumers are least likely to cut fresh meat consumption. Findings include implications for future livestock production and communication by the meat industry.
     NAL call no. HD1401.A56
     Descriptors: bovine spongiform encephalopathy, meat, fresh products, food safety, consumer behavior, consumption patterns, decision making, mass media, demography, consumer surveys, probit analysis, Belgium.

259. Von Holst, C., Honikel, K.O., Unglaub, W., Kramer, G., Anklam, Elke Determination of an appropriate heat treatment of animal waste using the ELISA technique: Results of a validation study. Meat Science. Jan. 2000. v. 54 (1) p. 1-7
     NAL call no. TX373.M4
     Descriptors: animal wastes, effective sterilisation determination, compound feed, inactivation, bovine spongiform encephalopathy, BSE, prion degradation, enzyme-linked immuno-sorbent assay, ELISA, test kit, statistical evaluation.

260. Walton, T. E. The impact of diseases on the importation of animals and animal products. Annals of the New York Academy of Sciences. 2000. 916: 36-40. ISSN: 0077-8923.
     Abstract: For decades the veterinary services of the United States and other nations protected their livestock and poultry industries from the ravages of introduced animal diseases by rigorous import restrictions. This policy of zero risk frequently translated to no or reduced trade in animals and animal products or dramatic trade inequities. However, GATT articles enforced by the WTO require that imported products be treated no less favorably than domestically produced goods with regard to animal health restrictions. Under authority from the WTO, the OIE establishes recommendations and guidelines for the regulation of trade in animals and products of animal origin through the OIE International Animal Health Code, sets animal health standards, and reports global animal health situations and statuses. Diseases often have a dramatic impact on the animal agricultural industries of a nation--disease outbreaks may be deleterious to the competitiveness of the products of one nation but offer opportunities for others. The potential dangers of lax vigilance, insufficient scientifically valid data, inadequate SPS measures, and errors in assessing risk can turn the heady experience of seemingly unlimited growth in international markets and demand for one's products into a catastrophic return to reality. The experience of the United Kingdom and countries of Europe with bovine spongiform encephalopathy is a case in point. It is estimated that the cost of the outbreak of this disease to the economy of the UK has been more than $3 billion. Responses of their trading partners, including the US, to this outbreak were abrupt and restrictive. Although the decision was controversial, the US stopped importation of live cattle from the UK in the late 1980's and subsequently, in 1997, importation of all products of ruminant origin was stopped from all countries of Europe. The transmission of the disease to continental Europe and the disclosure that the pathogen was associated with a fatal human illness, rocked consumer confidence in the safety of the UK beef supply, brought down the ruling political party in the UK, and forced major changes in the beef industry.
     NAL call no. 500 N484
     Descriptors: BSE, public health concerns, bovine-based products, impacts on trade.

261. Wang, R.-F., Myers, M.J., Campbellm W., Cao, W.-W., Painem D., Cerniglia, C.E. A rapid method for PCR detection of bovine materials in animal feedstuffs. Molecular and Cellular Probes. 2000. v. 14/1 p. 1-5
     Abstract: Rapid identification of bovine materials in animal feedstuffs is essential for effective control of a potential source of bovine spongiform encephalopathy. We have developed a rapid method for the detection of the presence of bovine materials in animal feeds. Animal feed samples were prepared by a Chelex-100 treatment method, then subjected to polymerase chain reaction (PCR) detection. The assay can be completed in 2 h including 30 min for sample preparation, 35-65 min for PCR cycling and 30 min for gel electrophoresis. This method is not only rapid, simple and consistent, but also avoids a hazardous waste disposal issue associated with a previously described guanidine thiocyanate (GuSCN) extraction-PCR method.
     NAL call no. RB43.7 M63
     Descriptors: polymerase chain reaction, PCR, BSE, bovine spongiform encephalopathy, identification of bovine-based materials, feed stuffs, methodologies, Chelex-100 treatment method, bovine tissue

262. Weber, E. L., Blanco Viera, J. F., Carrillo, B. J. Transmissible spongiform encephalopathies (TSE). 2. Aetiological aspects. [Encefalopatias espongiformes transmisibles (TSE). 2. Aspectos etiologicos.] Revista de Medicina Veterinaria (Buenos Aires). 2000. v. 81 (2) p. 143-144
     NAL call no. 41.8 B86
     Descriptors: prion diseases, etiology, prion proteins, pathogenesis, bovine spongiform encephalopathy, domestic animals, livestock, cattle, prions.

263. Wells, G.A.H., Bradley, R., Wilesmith, J.W. Bovine spongiform encephalopathy. Manual of standards for diagnostic tests and vaccines. List A and B diseases of mammals, birds and bees. 2000, Ed.4, 457-466, 67 ref. Office International des Epizooties, Paris, France ISBN: 92-9044-510-6
     NAL call no. SF771 M36 2000
     Descriptors: BSE, bovine spongiform encephalopathy, brain, cattle diseases, central nervous system, clinical aspects, diagnosis, diagnostic techniques, disease transmission, epidemiology, human diseases, immunodiagnosis, zoonoses

264. Wenisch, S., Lucker, E., Eigenbrodt, E., Bulte, M., Leiser, R. Procedures for the detection of unwanted ingredients in meat products with regard to BSE. 4. Histological and immunohistological procedures for the detection of central nervous system tissue in meat products. [Verfahren zum Nachweis von im Hinblick auf BSE unerwunschten Zutaten in Fleischerzeugnissen: 4. Histologischer und immunhistologischer Nachweis von Gehirn in Bruhwurst.] Fleischwirtschaft. 2000. v. 80 (7) p. 69-72
     NAL call no. 280.38 F62
     Descriptors: meat products, analytical detection methods, antibodies, bovine spongiform encephalopathy, brain, central nervous system, immunohistochemical procedure, monoclonal antibodies, muscles, nervous system, sausages, meat inspection, pigs.

265. Wernitznig, F. BSE und mikroskopischer Nachweis von "Tiermehl" im Mischfutter. [BSE and microscopic detection of "animal meal" in mixed feed.] Muhle + Mischfutter. 2000, 137: 26, 849-852. ISSN: 0027-2949. In German.
     NAL call no. 298.8 M89
     Descriptors: microscopic analysis, animal feed meals, muscle fibers, feather, hoof particles, fish-bone and animal bone fragments.

266. Wilesmith, J.W., Ryan, J.B.M., Stevenson, M.A., Morris, R.S., Pfeiffer, D.U., Lin, D., Jackson, R., Sanson, R.L. Temporal aspects of the epidemic of bovine spongiform encephalopathy in Great Britain: holding-associated risk factors for the disease. The Veterinary record: journal of the British Veterinary Association. London: The British Veterinary Association. Sep. 16, 2000. v. 147 (12) p. 319-325. Includes references.
     Abstract: The objectives of this study were first to describe the pattern of the epidemic of bovine spongiform encephalopathy (BSE) in Great Britain in terms of the temporal change in the proportion of all cattle holdings that had experienced at least one confirmed case of BSE to June 30, 1997, and secondly to identify risk factors that influenced the date of onset of a holding's first confirmed BSE case. The analyses were based on the population of British cattle at risk, derived from agricultural census data collected between 1986 and 1996, and the BSE case data collected up to June 30, 1997. The unit of interest was the cattle holding and included all those recorded at least once on annual agricultural censuses conducted between June 30, 1986, and June 30, 1996. The outcome of interest was the date on which clinical signs were recorded in a holding's first confirmed case of BSE, termed the BSE onset date. Univariate and multivariate survival analysis techniques were used to describe the temporal pattern of the epidemic. The BSE epidemic in Great Britain started in November 1986, with the majority of affected holdings having their BSE onset date after February 1992. After adjusting for the effect of the size and type of holding, holdings in the south of England (specifically those in the Eastern, South east and South west regions) had 2.22 to 2.43 (95 per cent confidence interval [CI] 2.07 to 2.58) times as great a monthly hazard of having a BSE index case as holdings in Scotland. After adjusting for the effect of region and type of holding, holdings with more than 53 adult cattle had 5.91 (95 per cent CI 5.62 to 6.21) times as great a monthly hazard of having a BSE index case as holdings with seven to 21 adult cattle. Dairy holdings had 3.06 (95 per cent CI 2.96 to 3.16) times as great a monthly hazard of having a BSE index case as beef suckler holdings. These analyses show that there were different rates of onset in different regions and in holdings of different sizes and types, that the epidemic was propagated most strongly in the south of the country, and that the growth of the epidemic followed essentially the same pattern in each region of the country, with modest temporal lags between them. The control measures imposed in 1988 and 1990 brought the expansion of the epidemic under control, although the rate of progress was slowed by those regions where the effectiveness of the control methods took some time to take full effect.
     NAL call no. 41.8 V641
     Descriptors: cattle, bovine spongiform encephalopathy, temporal variation, risk factors, epidemics, clinical aspects, disease control, Great Britain.

267. Wilks, C. The BSE inquiry and communication of risk. Aust Vet J. Brunswick, Vic. : Australian Veterinary Association, 1927-. Dec 2000. v. 78 (12) p. 837. ISSN: 0005-0423
     NAL call no. 41.8 Au72
     Descriptors: bovine spongiform encephalopathy, epidemics, reports, communication, UK.

268. Williams, E. S., Kirkwood, J. K., Miller, M.W., Williams, E.S. (ed.), Barker, I.K. Transmissible spongiform encephalopathies. In: Infectious Diseases of Wild Mammals. Iowa State University Press, Ames, USA, 2000, Ed. 3, 292-301, 105 ref. ISBN: 0-8138-2556-3
     NAL call no. SF996.4 I54 2000
     Descriptors: BSE, chronic wasting disease, etiology, bovine spongiform encephalopathy, clinical aspects, disease transmission, epidemiology, host-range, pathogenesis, transmissible spongiform encephalopathies, wild mammals.

269. Zaaijer, H. L. Boviene spongiforme encefalopathie en de veiligheid van voedsel. [Bovine spongiform encephalopathy and food safety]. Nederlands tijdschrift voor geneeskunde. May 27, 2000. 144(22): 1052-1057. ISSN: 0028-2162. In Dutch.
     Abstract: The governments of Great Britain and France disagree on the safety of British beef with respect to bovine spongiform encephalopathy (BSE). Eventually the consumer might have the burden to decide whether beef from Britain is safe to eat with regard to the new variant of Creutzfeldt-Jakob disease (vCJD). Outside Britain the incidence of BSE increases. Probably in continental Europe, the use of high risk material, derived from non-British cattle in the subclinical stage of BSE, poses a greater threat than beef imported from Great Britain. The risk to contract vCJD depends on two unknown factors: the susceptibility of man to BSE and the amount of success in keeping infectious tissue of BSE-incubating cattle out of the human food chain. Until the susceptibility of humans to BSE and the extent to which our food is contaminated become known, no clear-cut advice can be given on the safety of certain food items. There appears to be a genetic predisposition to vCJD, i.e. methionine homozygosity at codon 129 of the normal human prion protein gene. The European Community should ban the use of any high risk material for production of food, except in production processes for which inactivation of the BSE agent has been proven. The facts that are known at this moment allow the conclusion that gelatin and beef products of unknown origin and composition pose a greater health threat than eating genuine beef (muscle tissue).
     Descriptors: BSE, NvCJD, risks of eating beef, epidemiology, bovine-based products, public health risks, UK, Europe.

270. Zaaijer, H.L. Confusion surrounding bovine spongiform encephalopathy (BSE) and the risk of variant Creutzfeldt-Jakob disease. [Verwarring rond boviene spongiforme encefalopathie (BSE) en her risico op de nieuwe variant van de ziekte van Creutzfeldt-Jakob.] Nederlands Tijdschrift voor Geneeskunde. Nov. 25, 2000. v. 144 (48) p. 2288-2290
     Abstract: There is a lot of confusing news regarding the risks of consuming beef for contracting variant Creutzfeldt-Jakob disease. Bureaucratic inertia and political expediency are fueled by the lack of pathogenetic and epidemiologic understanding of the mode of transmission. Consumers discard beef from their diet, which may be the least contaminated tissue, but other meat products, of which the risks are probably much higher, continue to enjoy free international trade and may be used in the human diet. British beef may be less harmful than French sausage. In addition, criminal and fraudulent practices pose a considerable threat to which an appropriate political answer has yet to be formulated.
     Descriptors: BSE, disease transmission risks, transmissible spongiform encephalopathies, Nv Creutzfeldt-Jakob disease, meat and meat product safety concerns.

271. Zeidler, M., Ironside, J.W. The new variant of Creutzfeldt-Jakob Disease. Revue Scientifique et Technique Office International des Epizooties. April 2000. v. 19 (1) p. 98-120
     Abstract: New variant Creutzfeldt-Jakob Disease (NvCJD) is a novel human transmissible spongiform encephalopathy first identified in 1996 in the United Kingdom (UK). Subsequent scientific studies revealed the strain of the transmissible agent responsible for NvCJD is identical to the bovine spongiform encephalopathy (BSE) agent. The disease has been considered as 'human BSE'. By December 31, 1999, 52 cases of NvCJD had been reported (49 cases in the UK, two cases in France and one case in the Republic of Ireland). All these individuals were under 53 years of age and all those tested were methionine homozygotes at codon 129 of the prion protein gene. A projection in the number of cases of NvCJD likely to occur in the future is impossible to estimate because of multiple uncertainties. These include the disease incubation period, the degree of exposure to the infective agent and the susceptibility of other genetic subtypes. Continued surveillance of both BSE and CJD is required in the UK and in other countries, to ensure that the potential epidemic is adequately monitored and all possible steps are taken to prevent further human exposure to the BSE agent.
     NAL call no. SF781 R4
     Descriptors: NvCreutzfeldt-Jakob Disease, NvCJD, bovine spongiform encephalopathy agent, BSE, variant typing, United Kingdon, France, Republic of Ireland, prion protein gene, epidemiological factors, risk factors.

272. Zwingmann W. Bovine spongiform encephalopathy in Germany; Foot and mouth disease in Swaziland and South Africa; Lumpy skin disease in Mauritius; Newcastle disease in Zambia. Disease Information Office International des Epizooties. 2000, 13: 49, 8 pp. ISSN: 1012-5329
     NAL call no. SF781 D57
     Descriptors: BSE, bovine spongiform encephalopathy, foot and mouth disease, Newcastle disease, disease surveys, Africa.