About
Site Map
Contact Us
A service of the
U.S. National Library of Medicine®
Home
Conditions
Genes
Chromosomes
Handbook
Glossary
Resources
HTT Entrez Gene digest
>
GeneRIFs
Huntingtin
Entrez Gene GeneRIFs are recent publications from the PubMed® catalog that contain relevant information about HTT.
Anne SL, Saudou F, Humbert S. Phosphorylation of huntingtin by cyclin-dependent kinase 5 is induced by DNA damage and regulates wild-type and mutant huntingtin toxicity in neurons. J Neurosci. 2007 Jul 4;27(27):7318-28.
PubMed citation
Aziz NA, van der Burg JM, Landwehrmeyer GB, Brundin P, Stijnen T, EHDI Study Group, Roos RA. Weight loss in Huntington disease increases with higher CAG repeat number. Neurology. 2008 Nov 4;71(19):1506-13.
PubMed citation
Bae BI, Xu H, Igarashi S, Fujimuro M, Agrawal N, Taya Y, Hayward SD, Moran TH, Montell C, Ross CA, Snyder SH, Sawa A. p53 mediates cellular dysfunction and behavioral abnormalities in Huntington's disease. Neuron. 2005 Jul 7;47(1):29-41.
PubMed citation
Banoei MM, Houshmand M, Panahi MS, Shariati P, Rostami M, Manshadi MD, Majidizadeh T. Huntington's disease and mitochondrial DNA deletions: event or regular mechanism for mutant huntingtin protein and CAG repeats expansion?! Cell Mol Neurobiol. 2007 Nov;27(7):867-75. Epub 2007 Oct 20.
PubMed citation
Behrens PF, Franz P, Woodman B, Lindenberg KS, Landwehrmeyer GB. Impaired glutamate transport and glutamate-glutamine cycling: downstream effects of the Huntington mutation. Brain. 2002 Aug;125(Pt 8):1908-22.
PubMed citation
Benchoua A, Trioulier Y, Diguet E, Malgorn C, Gaillard MC, Dufour N, Elalouf JM, Krajewski S, Hantraye P, Déglon N, Brouillet E. Dopamine determines the vulnerability of striatal neurons to the N-terminal fragment of mutant huntingtin through the regulation of mitochondrial complex II. Hum Mol Genet. 2008 May 15;17(10):1446-56. Epub 2008 Feb 11.
PubMed citation
Benchoua A, Trioulier Y, Zala D, Gaillard MC, Lefort N, Dufour N, Saudou F, Elalouf JM, Hirsch E, Hantraye P, Déglon N, Brouillet E. Involvement of mitochondrial complex II defects in neuronal death produced by N-terminus fragment of mutated huntingtin. Mol Biol Cell. 2006 Apr;17(4):1652-63. Epub 2006 Feb 1.
PubMed citation
Benn CL, Landles C, Li H, Strand AD, Woodman B, Sathasivam K, Li SH, Ghazi-Noori S, Hockly E, Faruque SM, Cha JH, Sharpe PT, Olson JM, Li XJ, Bates GP. Contribution of nuclear and extranuclear polyQ to neurological phenotypes in mouse models of Huntington's disease. Hum Mol Genet. 2005 Oct 15;14(20):3065-78. Epub 2005 Sep 23.
PubMed citation
Bjørkøy G, Lamark T, Brech A, Outzen H, Perander M, Overvatn A, Stenmark H, Johansen T. p62/SQSTM1 forms protein aggregates degraded by autophagy and has a protective effect on huntingtin-induced cell death. J Cell Biol. 2005 Nov 21;171(4):603-14. Epub 2005 Nov 14.
PubMed citation
Blanco S, Suarez A, Gandia-Pla S, Gomez-Llorente C, Antunez A, Gomez-Capilla JA, Farez-Vidal ME. Use of capillary electrophoresis for accurate determination of CAG repeats causing Huntington disease. An oligonucleotide design avoiding shadow bands. Scand J Clin Lab Invest. 2008 Apr 9;:1-8 [Epub ahead of print].
PubMed citation
Boutell JM, Thomas P, Neal JW, Weston VJ, Duce J, Harper PS, Jones AL. Aberrant interactions of transcriptional repressor proteins with the Huntington's disease gene product, huntingtin. Hum Mol Genet. 1999 Sep;8(9):1647-55.
PubMed citation
Boutell JM, Thomas P, Neal JW, Weston VJ, Duce J, Harper PS, Jones AL. Aberrant interactions of transcriptional repressor proteins with the Huntington's disease gene product, huntingtin. Hum Mol Genet. 1999 Sep;8(9):1647-55.
PubMed citation
Boutell JM, Thomas P, Neal JW, Weston VJ, Duce J, Harper PS, Jones AL. Aberrant interactions of transcriptional repressor proteins with the Huntington's disease gene product, huntingtin. Hum Mol Genet. 1999 Sep;8(9):1647-55.
PubMed citation
Boutell JM, Thomas P, Neal JW, Weston VJ, Duce J, Harper PS, Jones AL. Aberrant interactions of transcriptional repressor proteins with the Huntington's disease gene product, huntingtin. Hum Mol Genet. 1999 Sep;8(9):1647-55.
PubMed citation
Boutell JM, Thomas P, Neal JW, Weston VJ, Duce J, Harper PS, Jones AL. Aberrant interactions of transcriptional repressor proteins with the Huntington's disease gene product, huntingtin. Hum Mol Genet. 1999 Sep;8(9):1647-55.
PubMed citation
Brown TB, Bogush AI, Ehrlich ME. Neocortical expression of mutant huntingtin is not required for alterations in striatal gene expression or motor dysfunction in a transgenic mouse. Hum Mol Genet. 2008 Oct 15;17(20):3095-104. Epub 2008 Jul 16.
PubMed citation
Burke JR, Enghild JJ, Martin ME, Jou YS, Myers RM, Roses AD, Vance JM, Strittmatter WJ. Huntingtin and DRPLA proteins selectively interact with the enzyme GAPDH. Nat Med. 1996 Mar;2(3):347-50.
PubMed citation
Burke JR, Enghild JJ, Martin ME, Jou YS, Myers RM, Roses AD, Vance JM, Strittmatter WJ. Huntingtin and DRPLA proteins selectively interact with the enzyme GAPDH. Nat Med. 1996 Mar;2(3):347-50.
PubMed citation
Burke MG, Woscholski R, Yaliraki SN. Differential hydrophobicity drives self-assembly in Huntington's disease. Proc Natl Acad Sci U S A. 2003 Nov 25;100(24):13928-33. Epub 2003 Nov 14.
PubMed citation
Carra S, Seguin SJ, Lambert H, Landry J. HspB8 chaperone activity toward poly(Q)-containing proteins depends on its association with Bag3, a stimulator of macroautophagy. J Biol Chem. 2008 Jan 18;283(3):1437-44. Epub 2007 Nov 15.
PubMed citation
Caviston JP, Ross JL, Antony SM, Tokito M, Holzbaur EL. Huntingtin facilitates dynein/dynactin-mediated vesicle transport. Proc Natl Acad Sci U S A. 2007 Jun 12;104(24):10045-50. Epub 2007 Jun 4.
PubMed citation
Chandra S, Shao J, Li JX, Li M, Longo FM, Diamond MI. A common motif targets huntingtin and the androgen receptor to the proteasome. J Biol Chem. 2008 Aug 29;283(35):23950-5. Epub 2008 Jun 27.
PubMed citation
Chang DT, Rintoul GL, Pandipati S, Reynolds IJ. Mutant huntingtin aggregates impair mitochondrial movement and trafficking in cortical neurons. Neurobiol Dis. 2006 May;22(2):388-400. Epub 2006 Feb 9.
PubMed citation
Chiang MC, Lee YC, Huang CL, Chern Y. cAMP-response element-binding protein contributes to suppression of the A2A adenosine receptor promoter by mutant Huntingtin with expanded polyglutamine residues. J Biol Chem. 2005 Apr 8;280(14):14331-40. Epub 2005 Feb 2.
PubMed citation
Choo YS, Johnson GV, MacDonald M, Detloff PJ, Lesort M. Mutant huntingtin directly increases susceptibility of mitochondria to the calcium-induced permeability transition and cytochrome c release. Hum Mol Genet. 2004 Jul 15;13(14):1407-20. Epub 2004 May 26.
PubMed citation
Chopra VS, Metzler M, Rasper DM, Engqvist-Goldstein AE, Singaraja R, Gan L, Fichter KM, McCutcheon K, Drubin D, Nicholson DW, Hayden MR. HIP12 is a non-proapoptotic member of a gene family including HIP1, an interacting protein with huntingtin. Mamm Genome. 2000 Nov;11(11):1006-15.
PubMed citation
Chopra VS, Metzler M, Rasper DM, Engqvist-Goldstein AE, Singaraja R, Gan L, Fichter KM, McCutcheon K, Drubin D, Nicholson DW, Hayden MR. HIP12 is a non-proapoptotic member of a gene family including HIP1, an interacting protein with huntingtin. Mamm Genome. 2000 Nov;11(11):1006-15.
PubMed citation
Chopra VS, Metzler M, Rasper DM, Engqvist-Goldstein AE, Singaraja R, Gan L, Fichter KM, McCutcheon K, Drubin D, Nicholson DW, Hayden MR. HIP12 is a non-proapoptotic member of a gene family including HIP1, an interacting protein with huntingtin. Mamm Genome. 2000 Nov;11(11):1006-15.
PubMed citation
Chopra VS, Metzler M, Rasper DM, Engqvist-Goldstein AE, Singaraja R, Gan L, Fichter KM, McCutcheon K, Drubin D, Nicholson DW, Hayden MR. HIP12 is a non-proapoptotic member of a gene family including HIP1, an interacting protein with huntingtin. Mamm Genome. 2000 Nov;11(11):1006-15.
PubMed citation
Chopra VS, Metzler M, Rasper DM, Engqvist-Goldstein AE, Singaraja R, Gan L, Fichter KM, McCutcheon K, Drubin D, Nicholson DW, Hayden MR. HIP12 is a non-proapoptotic member of a gene family including HIP1, an interacting protein with huntingtin. Mamm Genome. 2000 Nov;11(11):1006-15.
PubMed citation
Chun W, Lesort M, Tucholski J, Faber PW, MacDonald ME, Ross CA, Johnson GV. Tissue transglutaminase selectively modifies proteins associated with truncated mutant huntingtin in intact cells. Neurobiol Dis. 2001 Jun;8(3):391-404.
PubMed citation
Chun W, Lesort M, Tucholski J, Faber PW, MacDonald ME, Ross CA, Johnson GV. Tissue transglutaminase selectively modifies proteins associated with truncated mutant huntingtin in intact cells. Neurobiol Dis. 2001 Jun;8(3):391-404.
PubMed citation
Ciammola A, Sassone J, Alberti L, Meola G, Mancinelli E, Russo MA, Squitieri F, Silani V. Increased apoptosis, Huntingtin inclusions and altered differentiation in muscle cell cultures from Huntington's disease subjects. Cell Death Differ. 2006 Dec;13(12):2068-78. Epub 2006 May 26.
PubMed citation
Cong SY, Pepers BA, Evert BO, Rubinsztein DC, Roos RA, van Ommen GJ, Dorsman JC. Mutant huntingtin represses CBP, but not p300, by binding and protein degradation. Mol Cell Neurosci. 2005 Sep;30(1):12-23. Erratum in: Mol Cell Neurosci. 2005 Dec;30(4):559. Corrected and republished in: Mol Cell Neurosci. 2005 Dec;30(4):560-71.
PubMed citation
Cornett J, Cao F, Wang CE, Ross CA, Bates GP, Li SH, Li XJ. Polyglutamine expansion of huntingtin impairs its nuclear export. Nat Genet. 2005 Feb;37(2):198-204. Epub 2005 Jan 16.
PubMed citation
Cornett J, Cao F, Wang CE, Ross CA, Bates GP, Li SH, Li XJ. Polyglutamine expansion of huntingtin impairs its nuclear export. Nat Genet. 2005 Feb;37(2):198-204. Epub 2005 Jan 16.
PubMed citation
Cornett J, Cao F, Wang CE, Ross CA, Bates GP, Li SH, Li XJ. Polyglutamine expansion of huntingtin impairs its nuclear export. Nat Genet. 2005 Feb;37(2):198-204. Epub 2005 Jan 16.
PubMed citation
Cornett J, Cao F, Wang CE, Ross CA, Bates GP, Li SH, Li XJ. Polyglutamine expansion of huntingtin impairs its nuclear export. Nat Genet. 2005 Feb;37(2):198-204. Epub 2005 Jan 16.
PubMed citation
Cornett J, Smith L, Friedman M, Shin JY, Li XJ, Li SH. Context-dependent dysregulation of transcription by mutant huntingtin. J Biol Chem. 2006 Nov 24;281(47):36198-204. Epub 2006 Sep 29.
PubMed citation
Decruyenaere M, Evers-Kiebooms G, Cloostermans T, Boogaerts A, Demyttenaere K, Dom R, Fryns JP. Predictive testing for Huntington's disease: relationship with partners after testing. Clin Genet. 2004 Jan;65(1):24-31.
PubMed citation
del Toro D, Canals JM, Ginés S, Kojima M, Egea G, Alberch J. Mutant huntingtin impairs the post-Golgi trafficking of brain-derived neurotrophic factor but not its Val66Met polymorphism. J Neurosci. 2006 Dec 6;26(49):12748-57.
PubMed citation
Díaz-Hernández M, Moreno-Herrero F, Gómez-Ramos P, Morán MA, Ferrer I, Baró AM, Avila J, Hernández F, Lucas JJ. Biochemical, ultrastructural, and reversibility studies on huntingtin filaments isolated from mouse and human brain. J Neurosci. 2004 Oct 20;24(42):9361-71.
PubMed citation
Dunah AW, Jeong H, Griffin A, Kim YM, Standaert DG, Hersch SM, Mouradian MM, Young AB, Tanese N, Krainc D. Sp1 and TAFII130 transcriptional activity disrupted in early Huntington's disease. Science. 2002 Jun 21;296(5576):2238-43. Epub 2002 May 2.
PubMed citation
Dunah AW, Jeong H, Griffin A, Kim YM, Standaert DG, Hersch SM, Mouradian MM, Young AB, Tanese N, Krainc D. Sp1 and TAFII130 transcriptional activity disrupted in early Huntington's disease. Science. 2002 Jun 21;296(5576):2238-43. Epub 2002 May 2.
PubMed citation
Dunah AW, Jeong H, Griffin A, Kim YM, Standaert DG, Hersch SM, Mouradian MM, Young AB, Tanese N, Krainc D. Sp1 and TAFII130 transcriptional activity disrupted in early Huntington's disease. Science. 2002 Jun 21;296(5576):2238-43. Epub 2002 May 2.
PubMed citation
Edwardson JM, Wang CT, Gong B, Wyttenbach A, Bai J, Jackson MB, Chapman ER, Morton AJ. Expression of mutant huntingtin blocks exocytosis in PC12 cells by depletion of complexin II. J Biol Chem. 2003 Aug 15;278(33):30849-53. Epub 2003 Jun 13.
PubMed citation
Faber PW, Barnes GT, Srinidhi J, Chen J, Gusella JF, MacDonald ME. Huntingtin interacts with a family of WW domain proteins. Hum Mol Genet. 1998 Sep;7(9):1463-74.
PubMed citation
Faber PW, Barnes GT, Srinidhi J, Chen J, Gusella JF, MacDonald ME. Huntingtin interacts with a family of WW domain proteins. Hum Mol Genet. 1998 Sep;7(9):1463-74.
PubMed citation
Faber PW, Barnes GT, Srinidhi J, Chen J, Gusella JF, MacDonald ME. Huntingtin interacts with a family of WW domain proteins. Hum Mol Genet. 1998 Sep;7(9):1463-74.
PubMed citation
Faber PW, Barnes GT, Srinidhi J, Chen J, Gusella JF, MacDonald ME. Huntingtin interacts with a family of WW domain proteins. Hum Mol Genet. 1998 Sep;7(9):1463-74.
PubMed citation
Faber PW, Barnes GT, Srinidhi J, Chen J, Gusella JF, MacDonald ME. Huntingtin interacts with a family of WW domain proteins. Hum Mol Genet. 1998 Sep;7(9):1463-74.
PubMed citation
Faber PW, Barnes GT, Srinidhi J, Chen J, Gusella JF, MacDonald ME. Huntingtin interacts with a family of WW domain proteins. Hum Mol Genet. 1998 Sep;7(9):1463-74.
PubMed citation
Faber PW, Barnes GT, Srinidhi J, Chen J, Gusella JF, MacDonald ME. Huntingtin interacts with a family of WW domain proteins. Hum Mol Genet. 1998 Sep;7(9):1463-74.
PubMed citation
Faber PW, Barnes GT, Srinidhi J, Chen J, Gusella JF, MacDonald ME. Huntingtin interacts with a family of WW domain proteins. Hum Mol Genet. 1998 Sep;7(9):1463-74.
PubMed citation
Faber PW, Barnes GT, Srinidhi J, Chen J, Gusella JF, MacDonald ME. Huntingtin interacts with a family of WW domain proteins. Hum Mol Genet. 1998 Sep;7(9):1463-74.
PubMed citation
Faber PW, Barnes GT, Srinidhi J, Chen J, Gusella JF, MacDonald ME. Huntingtin interacts with a family of WW domain proteins. Hum Mol Genet. 1998 Sep;7(9):1463-74.
PubMed citation
Faber PW, Barnes GT, Srinidhi J, Chen J, Gusella JF, MacDonald ME. Huntingtin interacts with a family of WW domain proteins. Hum Mol Genet. 1998 Sep;7(9):1463-74.
PubMed citation
Faber PW, Barnes GT, Srinidhi J, Chen J, Gusella JF, MacDonald ME. Huntingtin interacts with a family of WW domain proteins. Hum Mol Genet. 1998 Sep;7(9):1463-74.
PubMed citation
Faber PW, Barnes GT, Srinidhi J, Chen J, Gusella JF, MacDonald ME. Huntingtin interacts with a family of WW domain proteins. Hum Mol Genet. 1998 Sep;7(9):1463-74.
PubMed citation
Faber PW, Barnes GT, Srinidhi J, Chen J, Gusella JF, MacDonald ME. Huntingtin interacts with a family of WW domain proteins. Hum Mol Genet. 1998 Sep;7(9):1463-74.
PubMed citation
Faber PW, Barnes GT, Srinidhi J, Chen J, Gusella JF, MacDonald ME. Huntingtin interacts with a family of WW domain proteins. Hum Mol Genet. 1998 Sep;7(9):1463-74.
PubMed citation
Faber PW, Barnes GT, Srinidhi J, Chen J, Gusella JF, MacDonald ME. Huntingtin interacts with a family of WW domain proteins. Hum Mol Genet. 1998 Sep;7(9):1463-74.
PubMed citation
Faber PW, Barnes GT, Srinidhi J, Chen J, Gusella JF, MacDonald ME. Huntingtin interacts with a family of WW domain proteins. Hum Mol Genet. 1998 Sep;7(9):1463-74.
PubMed citation
Faber PW, Barnes GT, Srinidhi J, Chen J, Gusella JF, MacDonald ME. Huntingtin interacts with a family of WW domain proteins. Hum Mol Genet. 1998 Sep;7(9):1463-74.
PubMed citation
Faber PW, Barnes GT, Srinidhi J, Chen J, Gusella JF, MacDonald ME. Huntingtin interacts with a family of WW domain proteins. Hum Mol Genet. 1998 Sep;7(9):1463-74.
PubMed citation
Feng Z, Jin S, Zupnick A, Hoh J, de Stanchina E, Lowe S, Prives C, Levine AJ. p53 tumor suppressor protein regulates the levels of huntingtin gene expression. Oncogene. 2006 Jan 5;25(1):1-7.
PubMed citation
Gao YG, Yan XZ, Song AX, Chang YG, Gao XC, Jiang N, Zhang Q, Hu HY. Structural insights into the specific binding of huntingtin proline-rich region with the SH3 and WW domains. Structure. 2006 Dec;14(12):1755-65.
PubMed citation
Garcia M, Charvin D, Caboche J. Expanded huntingtin activates the c-Jun terminal kinase/c-Jun pathway prior to aggregate formation in striatal neurons in culture. Neuroscience. 2004;127(4):859-70.
PubMed citation
Gervais FG, Singaraja R, Xanthoudakis S, Gutekunst CA, Leavitt BR, Metzler M, Hackam AS, Tam J, Vaillancourt JP, Houtzager V, Rasper DM, Roy S, Hayden MR, Nicholson DW. Recruitment and activation of caspase-8 by the Huntingtin-interacting protein Hip-1 and a novel partner Hippi. Nat Cell Biol. 2002 Feb;4(2):95-105.
PubMed citation
Gil JM, Mohapel P, Araújo IM, Popovic N, Li JY, Brundin P, Petersén A. Reduced hippocampal neurogenesis in R6/2 transgenic Huntington's disease mice. Neurobiol Dis. 2005 Dec;20(3):744-51. Epub 2005 Jun 13.
PubMed citation
Goehler H, Lalowski M, Stelzl U, Waelter S, Stroedicke M, Worm U, Droege A, Lindenberg KS, Knoblich M, Haenig C, Herbst M, Suopanki J, Scherzinger E, Abraham C, Bauer B, Hasenbank R, Fritzsche A, Ludewig AH, Büssow K, Coleman SH, Gutekunst CA, Landwehrmeyer BG, Lehrach H, Wanker EE. A protein interaction network links GIT1, an enhancer of huntingtin aggregation, to Huntington's disease. Mol Cell. 2004 Sep 24;15(6):853-65. Erratum in: Mol Cell. 2005 Jul 22;19(2):287. Buessow, Konrad [corrected to Bussow, Konrad].
PubMed citation
González-González MC, Trujillo MJ, Rodríguez de Alba M, Ramos C. Early Huntington disease prenatal diagnosis by maternal semiquantitative fluorescent-PCR. Neurology. 2003 Apr 8;60(7):1214-5. No abstract available.
PubMed citation
Graham RK, Deng Y, Slow EJ, Haigh B, Bissada N, Lu G, Pearson J, Shehadeh J, Bertram L, Murphy Z, Warby SC, Doty CN, Roy S, Wellington CL, Leavitt BR, Raymond LA, Nicholson DW, Hayden MR. Cleavage at the caspase-6 site is required for neuronal dysfunction and degeneration due to mutant huntingtin. Cell. 2006 Jun 16;125(6):1179-91.
PubMed citation
Hattula K, Peränen J. FIP-2, a coiled-coil protein, links Huntingtin to Rab8 and modulates cellular morphogenesis. Curr Biol. 2000 Dec 14-28;10(24):1603-6.
PubMed citation
Hattula K, Peränen J. FIP-2, a coiled-coil protein, links Huntingtin to Rab8 and modulates cellular morphogenesis. Curr Biol. 2000 Dec 14-28;10(24):1603-6.
PubMed citation
Hattula K, Peränen J. FIP-2, a coiled-coil protein, links Huntingtin to Rab8 and modulates cellular morphogenesis. Curr Biol. 2000 Dec 14-28;10(24):1603-6.
PubMed citation
Hermel E, Gafni J, Propp SS, Leavitt BR, Wellington CL, Young JE, Hackam AS, Logvinova AV, Peel AL, Chen SF, Hook V, Singaraja R, Krajewski S, Goldsmith PC, Ellerby HM, Hayden MR, Bredesen DE, Ellerby LM. Specific caspase interactions and amplification are involved in selective neuronal vulnerability in Huntington's disease. Cell Death Differ. 2004 Apr;11(4):424-38.
PubMed citation
Hermel E, Gafni J, Propp SS, Leavitt BR, Wellington CL, Young JE, Hackam AS, Logvinova AV, Peel AL, Chen SF, Hook V, Singaraja R, Krajewski S, Goldsmith PC, Ellerby HM, Hayden MR, Bredesen DE, Ellerby LM. Specific caspase interactions and amplification are involved in selective neuronal vulnerability in Huntington's disease. Cell Death Differ. 2004 Apr;11(4):424-38.
PubMed citation
Hermel E, Gafni J, Propp SS, Leavitt BR, Wellington CL, Young JE, Hackam AS, Logvinova AV, Peel AL, Chen SF, Hook V, Singaraja R, Krajewski S, Goldsmith PC, Ellerby HM, Hayden MR, Bredesen DE, Ellerby LM. Specific caspase interactions and amplification are involved in selective neuronal vulnerability in Huntington's disease. Cell Death Differ. 2004 Apr;11(4):424-38.
PubMed citation
Hermel E, Gafni J, Propp SS, Leavitt BR, Wellington CL, Young JE, Hackam AS, Logvinova AV, Peel AL, Chen SF, Hook V, Singaraja R, Krajewski S, Goldsmith PC, Ellerby HM, Hayden MR, Bredesen DE, Ellerby LM. Specific caspase interactions and amplification are involved in selective neuronal vulnerability in Huntington's disease. Cell Death Differ. 2004 Apr;11(4):424-38.
PubMed citation
Hoffner G, Kahlem P, Djian P. Perinuclear localization of huntingtin as a consequence of its binding to microtubules through an interaction with beta-tubulin: relevance to Huntington's disease. J Cell Sci. 2002 Mar 1;115(Pt 5):941-8.
PubMed citation
Hoffner G, Kahlem P, Djian P. Perinuclear localization of huntingtin as a consequence of its binding to microtubules through an interaction with beta-tubulin: relevance to Huntington's disease. J Cell Sci. 2002 Mar 1;115(Pt 5):941-8.
PubMed citation
Holbert S, Dedeoglu A, Humbert S, Saudou F, Ferrante RJ, Néri C. Cdc42-interacting protein 4 binds to huntingtin: neuropathologic and biological evidence for a role in Huntington's disease. Proc Natl Acad Sci U S A. 2003 Mar 4;100(5):2712-7. Epub 2003 Feb 25.
PubMed citation
Holbert S, Dedeoglu A, Humbert S, Saudou F, Ferrante RJ, Néri C. Cdc42-interacting protein 4 binds to huntingtin: neuropathologic and biological evidence for a role in Huntington's disease. Proc Natl Acad Sci U S A. 2003 Mar 4;100(5):2712-7. Epub 2003 Feb 25.
PubMed citation
Holbert S, Dedeoglu A, Humbert S, Saudou F, Ferrante RJ, Néri C. Cdc42-interacting protein 4 binds to huntingtin: neuropathologic and biological evidence for a role in Huntington's disease. Proc Natl Acad Sci U S A. 2003 Mar 4;100(5):2712-7. Epub 2003 Feb 25.
PubMed citation
Holbert S, Denghien I, Kiechle T, Rosenblatt A, Wellington C, Hayden MR, Margolis RL, Ross CA, Dausset J, Ferrante RJ, Néri C. The Gln-Ala repeat transcriptional activator CA150 interacts with huntingtin: neuropathologic and genetic evidence for a role in Huntington's disease pathogenesis. Proc Natl Acad Sci U S A. 2001 Feb 13;98(4):1811-6. Epub 2001 Jan 30.
PubMed citation
Holbert S, Denghien I, Kiechle T, Rosenblatt A, Wellington C, Hayden MR, Margolis RL, Ross CA, Dausset J, Ferrante RJ, Néri C. The Gln-Ala repeat transcriptional activator CA150 interacts with huntingtin: neuropathologic and genetic evidence for a role in Huntington's disease pathogenesis. Proc Natl Acad Sci U S A. 2001 Feb 13;98(4):1811-6. Epub 2001 Jan 30.
PubMed citation
Holbert S, Denghien I, Kiechle T, Rosenblatt A, Wellington C, Hayden MR, Margolis RL, Ross CA, Dausset J, Ferrante RJ, Néri C. The Gln-Ala repeat transcriptional activator CA150 interacts with huntingtin: neuropathologic and genetic evidence for a role in Huntington's disease pathogenesis. Proc Natl Acad Sci U S A. 2001 Feb 13;98(4):1811-6. Epub 2001 Jan 30.
PubMed citation
Holbert S, Denghien I, Kiechle T, Rosenblatt A, Wellington C, Hayden MR, Margolis RL, Ross CA, Dausset J, Ferrante RJ, Néri C. The Gln-Ala repeat transcriptional activator CA150 interacts with huntingtin: neuropathologic and genetic evidence for a role in Huntington's disease pathogenesis. Proc Natl Acad Sci U S A. 2001 Feb 13;98(4):1811-6. Epub 2001 Jan 30.
PubMed citation
Holbert S, Denghien I, Kiechle T, Rosenblatt A, Wellington C, Hayden MR, Margolis RL, Ross CA, Dausset J, Ferrante RJ, Néri C. The Gln-Ala repeat transcriptional activator CA150 interacts with huntingtin: neuropathologic and genetic evidence for a role in Huntington's disease pathogenesis. Proc Natl Acad Sci U S A. 2001 Feb 13;98(4):1811-6. Epub 2001 Jan 30.
PubMed citation
Hollán S, Vécsei L, Magyar K. Adverse effects of dopamine potentiation by long-term treatment with selegiline. Mov Disord. 2004 Jan;19(1):107-9.
PubMed citation
Ho LW, Brown R, Maxwell M, Wyttenbach A, Rubinsztein DC. Wild type Huntingtin reduces the cellular toxicity of mutant Huntingtin in mammalian cell models of Huntington's disease. J Med Genet. 2001 Jul;38(7):450-2.
PubMed citation
Horn SC, Lalowski M, Goehler H, Dröge A, Wanker EE, Stelzl U. Huntingtin interacts with the receptor sorting family protein GASP2. J Neural Transm. 2006 Aug;113(8):1081-90. Epub 2006 Jul 13.
PubMed citation
Huang B, Kochanek S. Adenovirus-mediated silencing of huntingtin expression by shRNA. Hum Gene Ther. 2005 May;16(5):618-26.
PubMed citation
Huang CC, Faber PW, Persichetti F, Mittal V, Vonsattel JP, MacDonald ME, Gusella JF. Amyloid formation by mutant huntingtin: threshold, progressivity and recruitment of normal polyglutamine proteins. Somat Cell Mol Genet. 1998 Jul;24(4):217-33.
PubMed citation
Huang K, Yanai A, Kang R, Arstikaitis P, Singaraja RR, Metzler M, Mullard A, Haigh B, Gauthier-Campbell C, Gutekunst CA, Hayden MR, El-Husseini A. Huntingtin-interacting protein HIP14 is a palmitoyl transferase involved in palmitoylation and trafficking of multiple neuronal proteins. Neuron. 2004 Dec 16;44(6):977-86.
PubMed citation
Hu H, McCaw EA, Hebb AL, Gomez GT, Denovan-Wright EM. Mutant huntingtin affects the rate of transcription of striatum-specific isoforms of phosphodiesterase 10A. Eur J Neurosci. 2004 Dec;20(12):3351-63.
PubMed citation
Humbert S, Bryson EA, Cordelières FP, Connors NC, Datta SR, Finkbeiner S, Greenberg ME, Saudou F. The IGF-1/Akt pathway is neuroprotective in Huntington's disease and involves Huntingtin phosphorylation by Akt. Dev Cell. 2002 Jun;2(6):831-7.
PubMed citation
Humbert S, Bryson EA, Cordelières FP, Connors NC, Datta SR, Finkbeiner S, Greenberg ME, Saudou F. The IGF-1/Akt pathway is neuroprotective in Huntington's disease and involves Huntingtin phosphorylation by Akt. Dev Cell. 2002 Jun;2(6):831-7.
PubMed citation
Iwata A, Riley BE, Johnston JA, Kopito RR. HDAC6 and microtubules are required for autophagic degradation of aggregated huntingtin. J Biol Chem. 2005 Dec 2;280(48):40282-92. Epub 2005 Sep 28.
PubMed citation
Jones AL. The localization and interactions of huntingtin. Philos Trans R Soc Lond B Biol Sci. 1999 Jun 29;354(1386):1021-7. Review.
PubMed citation
Kalchman MA, Graham RK, Xia G, Koide HB, Hodgson JG, Graham KC, Goldberg YP, Gietz RD, Pickart CM, Hayden MR. Huntingtin is ubiquitinated and interacts with a specific ubiquitin-conjugating enzyme. J Biol Chem. 1996 Aug 9;271(32):19385-94.
PubMed citation
Kalchman MA, Graham RK, Xia G, Koide HB, Hodgson JG, Graham KC, Goldberg YP, Gietz RD, Pickart CM, Hayden MR. Huntingtin is ubiquitinated and interacts with a specific ubiquitin-conjugating enzyme. J Biol Chem. 1996 Aug 9;271(32):19385-94.
PubMed citation
Kaltenbach LS, Romero E, Becklin RR, Chettier R, Bell R, Phansalkar A, Strand A, Torcassi C, Savage J, Hurlburt A, Cha GH, Ukani L, Chepanoske CL, Zhen Y, Sahasrabudhe S, Olson J, Kurschner C, Ellerby LM, Peltier JM, Botas J, Hughes RE. Huntingtin interacting proteins are genetic modifiers of neurodegeneration. PLoS Genet. 2007 May 11;3(5):e82.
PubMed citation
Kaytor MD, Wilkinson KD, Warren ST. Modulating huntingtin half-life alters polyglutamine-dependent aggregate formation and cell toxicity. J Neurochem. 2004 May;89(4):962-73.
PubMed citation
Kegel KB, Meloni AR, Yi Y, Kim YJ, Doyle E, Cuiffo BG, Sapp E, Wang Y, Qin ZH, Chen JD, Nevins JR, Aronin N, DiFiglia M. Huntingtin is present in the nucleus, interacts with the transcriptional corepressor C-terminal binding protein, and represses transcription. J Biol Chem. 2002 Mar 1;277(9):7466-76. Epub 2001 Dec 5.
PubMed citation
Kegel KB, Meloni AR, Yi Y, Kim YJ, Doyle E, Cuiffo BG, Sapp E, Wang Y, Qin ZH, Chen JD, Nevins JR, Aronin N, DiFiglia M. Huntingtin is present in the nucleus, interacts with the transcriptional corepressor C-terminal binding protein, and represses transcription. J Biol Chem. 2002 Mar 1;277(9):7466-76. Epub 2001 Dec 5.
PubMed citation
Kegel KB, Meloni AR, Yi Y, Kim YJ, Doyle E, Cuiffo BG, Sapp E, Wang Y, Qin ZH, Chen JD, Nevins JR, Aronin N, DiFiglia M. Huntingtin is present in the nucleus, interacts with the transcriptional corepressor C-terminal binding protein, and represses transcription. J Biol Chem. 2002 Mar 1;277(9):7466-76. Epub 2001 Dec 5.
PubMed citation
Kegel KB, Meloni AR, Yi Y, Kim YJ, Doyle E, Cuiffo BG, Sapp E, Wang Y, Qin ZH, Chen JD, Nevins JR, Aronin N, DiFiglia M. Huntingtin is present in the nucleus, interacts with the transcriptional corepressor C-terminal binding protein, and represses transcription. J Biol Chem. 2002 Mar 1;277(9):7466-76. Epub 2001 Dec 5.
PubMed citation
Kegel KB, Sapp E, Yoder J, Cuiffo B, Sobin L, Kim YJ, Qin ZH, Hayden MR, Aronin N, Scott DL, Isenberg G, Goldmann WH, DiFiglia M. Huntingtin associates with acidic phospholipids at the plasma membrane. J Biol Chem. 2005 Oct 28;280(43):36464-73. Epub 2005 Aug 5.
PubMed citation
Kennedy L, Evans E, Chen CM, Craven L, Detloff PJ, Ennis M, Shelbourne PF. Dramatic tissue-specific mutation length increases are an early molecular event in Huntington disease pathogenesis. Hum Mol Genet. 2003 Dec 15;12(24):3359-67. Epub 2003 Oct 21.
PubMed citation
Kenney C, Powell S, Jankovic J. Autopsy-proven Huntington's disease with 29 trinucleotide repeats. Mov Disord. 2007 Jan;22(1):127-30.
PubMed citation
Khoshnan A, Ko J, Patterson PH. Effects of intracellular expression of anti-huntingtin antibodies of various specificities on mutant huntingtin aggregation and toxicity. Proc Natl Acad Sci U S A. 2002 Jan 22;99(2):1002-7. Epub 2002 Jan 15.
PubMed citation
Kim MO, Chawla P, Overland RP, Xia E, Sadri-Vakili G, Cha JH. Altered histone monoubiquitylation mediated by mutant huntingtin induces transcriptional dysregulation. J Neurosci. 2008 Apr 9;28(15):3947-57.
PubMed citation
Kuhn A, Goldstein DR, Hodges A, Strand AD, Sengstag T, Kooperberg C, Becanovic K, Pouladi MA, Sathasivam K, Cha JH, Hannan AJ, Hayden MR, Leavitt BR, Dunnett SB, Ferrante RJ, Albin R, Shelbourne P, Delorenzi M, Augood SJ, Faull RL, Olson JM, Bates GP, Jones L, Luthi-Carter R. Mutant huntingtin's effects on striatal gene expression in mice recapitulate changes observed in human Huntington's disease brain and do not differ with mutant huntingtin length or wild-type huntingtin dosage. Hum Mol Genet. 2007 Aug 1;16(15):1845-61. Epub 2007 May 21.
PubMed citation
Leavitt BR, van Raamsdonk JM, Shehadeh J, Fernandes H, Murphy Z, Graham RK, Wellington CL, Raymond LA, Hayden MR. Wild-type huntingtin protects neurons from excitotoxicity. J Neurochem. 2006 Feb;96(4):1121-9. Epub 2006 Jan 17.
PubMed citation
Lee J, Park EH, Couture G, Harvey I, Garneau P, Pelletier J. An upstream open reading frame impedes translation of the huntingtin gene. Nucleic Acids Res. 2002 Dec 1;30(23):5110-9.
PubMed citation
Lee JM, Ivanova EV, Seong IS, Cashorali T, Kohane I, Gusella JF, MacDonald ME. Unbiased gene expression analysis implicates the huntingtin polyglutamine tract in extra-mitochondrial energy metabolism. PLoS Genet. 2007 Aug;3(8):e135. Epub 2007 Jun 27.
PubMed citation
Lee WC, Yoshihara M, Littleton JT. Cytoplasmic aggregates trap polyglutamine-containing proteins and block axonal transport in a Drosophila model of Huntington's disease. Proc Natl Acad Sci U S A. 2004 Mar 2;101(9):3224-9. Epub 2004 Feb 20.
PubMed citation
Li L, Murphy TH, Hayden MR, Raymond LA. Enhanced striatal NR2B-containing N-methyl-D-aspartate receptor-mediated synaptic currents in a mouse model of Huntington disease. J Neurophysiol. 2004 Nov;92(5):2738-46. Epub 2004 Jul 7.
PubMed citation
Li SH, Cheng AL, Zhou H, Lam S, Rao M, Li H, Li XJ. Interaction of Huntington disease protein with transcriptional activator Sp1. Mol Cell Biol. 2002 Mar;22(5):1277-87.
PubMed citation
Li SH, Cheng AL, Zhou H, Lam S, Rao M, Li H, Li XJ. Interaction of Huntington disease protein with transcriptional activator Sp1. Mol Cell Biol. 2002 Mar;22(5):1277-87.
PubMed citation
Li SH, Cheng AL, Zhou H, Lam S, Rao M, Li H, Li XJ. Interaction of Huntington disease protein with transcriptional activator Sp1. Mol Cell Biol. 2002 Mar;22(5):1277-87.
PubMed citation
Li SH, Gutekunst CA, Hersch SM, Li XJ. Interaction of huntingtin-associated protein with dynactin P150Glued. J Neurosci. 1998 Feb 15;18(4):1261-9.
PubMed citation
Li SH, Hosseini SH, Gutekunst CA, Hersch SM, Ferrante RJ, Li XJ. A human HAP1 homologue. Cloning, expression, and interaction with huntingtin. J Biol Chem. 1998 Jul 24;273(30):19220-7. Erratum in: J Biol Chem 1999 Apr 2;274(14):9906.
PubMed citation
Li SH, Hosseini SH, Gutekunst CA, Hersch SM, Ferrante RJ, Li XJ. A human HAP1 homologue. Cloning, expression, and interaction with huntingtin. J Biol Chem. 1998 Jul 24;273(30):19220-7. Erratum in: J Biol Chem 1999 Apr 2;274(14):9906.
PubMed citation
Li SH, Li XJ. Huntingtin and its role in neuronal degeneration. Neuroscientist. 2004 Oct;10(5):467-75. Review.
PubMed citation
Liu Y, Shen Y, Li H, Wang H, Yang ZR, Chen Y, Tang YP. Intergeneration CAG expansion in a Wuhan juvenile-onset Huntington disease family. Neurosci Bull. 2007 Jul;23(4):198-202.
PubMed citation
Liu YF, Deth RC, Devys D. SH3 domain-dependent association of huntingtin with epidermal growth factor receptor signaling complexes. J Biol Chem. 1997 Mar 28;272(13):8121-4.
PubMed citation
Liu YF, Deth RC, Devys D. SH3 domain-dependent association of huntingtin with epidermal growth factor receptor signaling complexes. J Biol Chem. 1997 Mar 28;272(13):8121-4.
PubMed citation
Liu YF, Deth RC, Devys D. SH3 domain-dependent association of huntingtin with epidermal growth factor receptor signaling complexes. J Biol Chem. 1997 Mar 28;272(13):8121-4.
PubMed citation
Liu YF, Deth RC, Devys D. SH3 domain-dependent association of huntingtin with epidermal growth factor receptor signaling complexes. J Biol Chem. 1997 Mar 28;272(13):8121-4.
PubMed citation
Liu YF, Deth RC, Devys D. SH3 domain-dependent association of huntingtin with epidermal growth factor receptor signaling complexes. J Biol Chem. 1997 Mar 28;272(13):8121-4.
PubMed citation
Liu YF, Deth RC, Devys D. SH3 domain-dependent association of huntingtin with epidermal growth factor receptor signaling complexes. J Biol Chem. 1997 Mar 28;272(13):8121-4.
PubMed citation
Liu YF, Deth RC, Devys D. SH3 domain-dependent association of huntingtin with epidermal growth factor receptor signaling complexes. J Biol Chem. 1997 Mar 28;272(13):8121-4.
PubMed citation
Liu YF, Deth RC, Devys D. SH3 domain-dependent association of huntingtin with epidermal growth factor receptor signaling complexes. J Biol Chem. 1997 Mar 28;272(13):8121-4.
PubMed citation
Liu YF, Dorow D, Marshall J. Activation of MLK2-mediated signaling cascades by polyglutamine-expanded huntingtin. J Biol Chem. 2000 Jun 23;275(25):19035-40.
PubMed citation
Liu YF, Dorow D, Marshall J. Activation of MLK2-mediated signaling cascades by polyglutamine-expanded huntingtin. J Biol Chem. 2000 Jun 23;275(25):19035-40.
PubMed citation
Liu YF, Dorow D, Marshall J. Activation of MLK2-mediated signaling cascades by polyglutamine-expanded huntingtin. J Biol Chem. 2000 Jun 23;275(25):19035-40.
PubMed citation
Li W, Serpell LC, Carter WJ, Rubinsztein DC, Huntington JA. Expression and characterization of full-length human huntingtin, an elongated HEAT repeat protein. J Biol Chem. 2006 Jun 9;281(23):15916-22. Epub 2006 Apr 4.
PubMed citation
Li XJ, Li SH, Sharp AH, Nucifora FC Jr, Schilling G, Lanahan A, Worley P, Snyder SH, Ross CA. A huntingtin-associated protein enriched in brain with implications for pathology. Nature. 1995 Nov 23;378(6555):398-402.
PubMed citation
Lunkes A, Lindenberg KS, Ben-Haïem L, Weber C, Devys D, Landwehrmeyer GB, Mandel JL, Trottier Y. Proteases acting on mutant huntingtin generate cleaved products that differentially build up cytoplasmic and nuclear inclusions. Mol Cell. 2002 Aug;10(2):259-69.
PubMed citation
Luo S, Mizuta H, Rubinsztein DC. p21-activated kinase 1 promotes soluble mutant huntingtin self-interaction and enhances toxicity. Hum Mol Genet. 2008 Mar 15;17(6):895-905. Epub 2007 Dec 7.
PubMed citation
Luo S, Vacher C, Davies JE, Rubinsztein DC. Cdk5 phosphorylation of huntingtin reduces its cleavage by caspases: implications for mutant huntingtin toxicity. J Cell Biol. 2005 May 23;169(4):647-56.
PubMed citation
Majumder P, Raychaudhuri S, Chattopadhyay B, Bhattacharyya NP. Increased caspase-2, calpain activations and decreased mitochondrial complex II activity in cells expressing exogenous huntingtin exon 1 containing CAG repeat in the pathogenic range. Cell Mol Neurobiol. 2007 Dec;27(8):1127-45. Epub 2007 Sep 28.
PubMed citation
Marcora E, Gowan K, Lee JE. Stimulation of NeuroD activity by huntingtin and huntingtin-associated proteins HAP1 and MLK2. Proc Natl Acad Sci U S A. 2003 Aug 5;100(16):9578-83. Epub 2003 Jul 24.
PubMed citation
Margolis RL, Holmes SE, Rosenblatt A, Gourley L, O'Hearn E, Ross CA, Seltzer WK, Walker RH, Ashizawa T, Rasmussen A, Hayden M, Almqvist EW, Harris J, Fahn S, MacDonald ME, Mysore J, Shimohata T, Tsuji S, Potter N, Nakaso K, Adachi Y, Nakashima K, Bird T, Krause A, Greenstein P. Huntington's Disease-like 2 (HDL2) in North America and Japan. Ann Neurol. 2004 Nov;56(5):670-4. Erratum in: Ann Neurol. 2004 Dec;56(6):911.
PubMed citation
Mihm MJ, Amann DM, Schanbacher BL, Altschuld RA, Bauer JA, Hoyt KR. Cardiac dysfunction in the R6/2 mouse model of Huntington's disease. Neurobiol Dis. 2007 Feb;25(2):297-308. Epub 2006 Nov 27.
PubMed citation
Milakovic T, Quintanilla RA, Johnson GV. Mutant huntingtin expression induces mitochondrial calcium handling defects in clonal striatal cells: functional consequences. J Biol Chem. 2006 Nov 17;281(46):34785-95. Epub 2006 Sep 13.
PubMed citation
Miller LC, Swayne LA, Chen L, Feng ZP, Wacker JL, Muchowski PJ, Zamponi GW, Braun JE. Cysteine string protein (CSP) inhibition of N-type calcium channels is blocked by mutant huntingtin. J Biol Chem. 2003 Dec 26;278(52):53072-81. Epub 2003 Oct 21.
PubMed citation
Miller SL, Scappini EL, O'Bryan J. Ubiquitin-interacting motifs inhibit aggregation of polyQ-expanded huntingtin. J Biol Chem. 2007 Mar 30;282(13):10096-103. Epub 2007 Feb 2.
PubMed citation
Modregger J, DiProspero NA, Charles V, Tagle DA, Plomann M. PACSIN 1 interacts with huntingtin and is absent from synaptic varicosities in presymptomatic Huntington's disease brains. Hum Mol Genet. 2002 Oct 1;11(21):2547-58.
PubMed citation
Modregger J, DiProspero NA, Charles V, Tagle DA, Plomann M. PACSIN 1 interacts with huntingtin and is absent from synaptic varicosities in presymptomatic Huntington's disease brains. Hum Mol Genet. 2002 Oct 1;11(21):2547-58.
PubMed citation
Modregger J, DiProspero NA, Charles V, Tagle DA, Plomann M. PACSIN 1 interacts with huntingtin and is absent from synaptic varicosities in presymptomatic Huntington's disease brains. Hum Mol Genet. 2002 Oct 1;11(21):2547-58.
PubMed citation
Modregger J, Schmidt AA, Ritter B, Huttner WB, Plomann M. Characterization of Endophilin B1b, a brain-specific membrane-associated lysophosphatidic acid acyl transferase with properties distinct from endophilin A1. J Biol Chem. 2003 Feb 7;278(6):4160-7. Epub 2002 Nov 26.
PubMed citation
Modregger J, Schmidt AA, Ritter B, Huttner WB, Plomann M. Characterization of Endophilin B1b, a brain-specific membrane-associated lysophosphatidic acid acyl transferase with properties distinct from endophilin A1. J Biol Chem. 2003 Feb 7;278(6):4160-7. Epub 2002 Nov 26.
PubMed citation
Morovvati S, Nakagawa M, Osame M, Karami A. Analysis of CCG repeats in Huntingtin gene among HD patients and normal populations in Japan. Arch Med Res. 2008 Jan;39(1):131-3. Epub 2007 Aug 23.
PubMed citation
Mugat B, Parmentier ML, Bonneaud N, Chan HY, Maschat F. Protective role of Engrailed in a Drosophila model of Huntington's disease. Hum Mol Genet. 2008 Nov 15;17(22):3601-16. Epub 2008 Aug 20.
PubMed citation
Myers RH. Huntington's disease genetics. NeuroRx. 2004 Apr;1(2):255-62. Review.
PubMed citation
Nasir J, Lafuente MJ, Duan K, Colomer V, Engelender S, Ingersoll R, Margolis RL, Ross CA, Hayden MR. Human huntingtin-associated protein (HAP-1) gene: genomic organisation and an intragenic polymorphism. Gene. 2000 Aug 22;254(1-2):181-7.
PubMed citation
Nucifora FC Jr, Sasaki M, Peters MF, Huang H, Cooper JK, Yamada M, Takahashi H, Tsuji S, Troncoso J, Dawson VL, Dawson TM, Ross CA. Interference by huntingtin and atrophin-1 with cbp-mediated transcription leading to cellular toxicity. Science. 2001 Mar 23;291(5512):2423-8.
PubMed citation
Nucifora FC Jr, Sasaki M, Peters MF, Huang H, Cooper JK, Yamada M, Takahashi H, Tsuji S, Troncoso J, Dawson VL, Dawson TM, Ross CA. Interference by huntingtin and atrophin-1 with cbp-mediated transcription leading to cellular toxicity. Science. 2001 Mar 23;291(5512):2423-8.
PubMed citation
Omi K, Hachiya NS, Tanaka M, Tokunaga K, Kaneko K. 14-3-3zeta is indispensable for aggregate formation of polyglutamine-expanded huntingtin protein. Neurosci Lett. 2008 Jan 24;431(1):45-50. Epub 2007 Nov 17.
PubMed citation
Orr AL, Li S, Wang CE, Li H, Wang J, Rong J, Xu X, Mastroberardino PG, Greenamyre JT, Li XJ. N-terminal mutant huntingtin associates with mitochondria and impairs mitochondrial trafficking. J Neurosci. 2008 Mar 12;28(11):2783-92.
PubMed citation
Paradisi I, Hernández A, Arias S. Huntington disease mutation in Venezuela: age of onset, haplotype analyses and geographic aggregation. J Hum Genet. 2008;53(2):127-35. Epub 2007 Dec 22. Erratum in: J Hum Genet. 2008;53(4):376.
PubMed citation
Park CW, Chen Z, Kren BT, Steer CJ. Double-stranded siRNA targeted to the huntingtin gene does not induce DNA methylation. Biochem Biophys Res Commun. 2004 Oct 8;323(1):275-80.
PubMed citation
Peel AL, Rao RV, Cottrell BA, Hayden MR, Ellerby LM, Bredesen DE. Double-stranded RNA-dependent protein kinase, PKR, binds preferentially to Huntington's disease (HD) transcripts and is activated in HD tissue. Hum Mol Genet. 2001 Jul 15;10(15):1531-8.
PubMed citation
Peters MF, Ross CA. Isolation of a 40-kDa Huntingtin-associated protein. J Biol Chem. 2001 Feb 2;276(5):3188-94. Epub 2000 Oct 16.
PubMed citation
Peters MF, Ross CA. Isolation of a 40-kDa Huntingtin-associated protein. J Biol Chem. 2001 Feb 2;276(5):3188-94. Epub 2000 Oct 16.
PubMed citation
Peters PJ, Ning K, Palacios F, Boshans RL, Kazantsev A, Thompson LM, Woodman B, Bates GP, D'Souza-Schorey C. Arfaptin 2 regulates the aggregation of mutant huntingtin protein. Nat Cell Biol. 2002 Mar;4(3):240-5.
PubMed citation
Petrasch-Parwez E, Nguyen HP, Löbbecke-Schumacher M, Habbes HW, Wieczorek S, Riess O, Andres KH, Dermietzel R, Von Hörsten S. Cellular and subcellular localization of Huntingtin [corrected] aggregates in the brain of a rat transgenic for Huntington disease. J Comp Neurol. 2007 Apr 10;501(5):716-30. Erratum in: J Comp Neurol. 2007 May 20;502(3):483.
PubMed citation
Ralser M, Nonhoff U, Albrecht M, Lengauer T, Wanker EE, Lehrach H, Krobitsch S. Ataxin-2 and huntingtin interact with endophilin-A complexes to function in plastin-associated pathways. Hum Mol Genet. 2005 Oct 1;14(19):2893-909. Epub 2005 Aug 22.
PubMed citation
Ramos-Arroyo MA, Moreno S, Valiente A. Incidence and mutation rates of Huntington's disease in Spain: experience of 9 years of direct genetic testing. J Neurol Neurosurg Psychiatry. 2005 Mar;76(3):337-42.
PubMed citation
Rangone H, Humbert S, Saudou F. Huntington's disease: how does huntingtin, an anti-apoptotic protein, become toxic? Pathol Biol (Paris). 2004 Jul;52(6):338-42. Review.
PubMed citation
Rangone H, Pardo R, Colin E, Girault JA, Saudou F, Humbert S. Phosphorylation of arfaptin 2 at Ser260 by Akt Inhibits PolyQ-huntingtin-induced toxicity by rescuing proteasome impairment. J Biol Chem. 2005 Jun 10;280(23):22021-8. Epub 2005 Apr 4.
PubMed citation
Raychaudhuri S, Sinha M, Mukhopadhyay D, Bhattacharyya NP. HYPK, a Huntingtin interacting protein, reduces aggregates and apoptosis induced by N-terminal Huntingtin with 40 glutamines in Neuro2a cells and exhibits chaperone-like activity. Hum Mol Genet. 2008 Jan 15;17(2):240-55. Epub 2007 Oct 18.
PubMed citation
Régulier E, Trottier Y, Perrin V, Aebischer P, Déglon N. Early and reversible neuropathology induced by tetracycline-regulated lentiviral overexpression of mutant huntingtin in rat striatum. Hum Mol Genet. 2003 Nov 1;12(21):2827-36. Epub 2003 Sep 2.
PubMed citation
Reijonen S, Putkonen N, Nørremølle A, Lindholm D, Korhonen L. Inhibition of endoplasmic reticulum stress counteracts neuronal cell death and protein aggregation caused by N-terminal mutant huntingtin proteins. Exp Cell Res. 2008 Mar 10;314(5):950-60. Epub 2008 Jan 14.
PubMed citation
Reiner A, Del Mar N, Deng YP, Meade CA, Sun Z, Goldowitz D. R6/2 neurons with intranuclear inclusions survive for prolonged periods in the brains of chimeric mice. J Comp Neurol. 2007 Dec 20;505(6):603-29.
PubMed citation
Rockabrand E, Slepko N, Pantalone A, Nukala VN, Kazantsev A, Marsh JL, Sullivan PG, Steffan JS, Sensi SL, Thompson LM. The first 17 amino acids of Huntingtin modulate its sub-cellular localization, aggregation and effects on calcium homeostasis. Hum Mol Genet. 2007 Jan 1;16(1):61-77. Epub 2006 Nov 29.
PubMed citation
Roze E, Betuing S, Deyts C, Marcon E, Brami-Cherrier K, Pagès C, Humbert S, Mérienne K, Caboche J. Mitogen- and stress-activated protein kinase-1 deficiency is involved in expanded-huntingtin-induced transcriptional dysregulation and striatal death. FASEB J. 2008 Apr;22(4):1083-93. Epub 2007 Nov 20.
PubMed citation
Ruan Q, Quintanilla RA, Johnson GV. Type 2 transglutaminase differentially modulates striatal cell death in the presence of wild type or mutant huntingtin. J Neurochem. 2007 Jul;102(1):25-36. Epub 2007 Mar 30.
PubMed citation
Sawa A, Nagata E, Sutcliffe S, Dulloor P, Cascio MB, Ozeki Y, Roy S, Ross CA, Snyder SH. Huntingtin is cleaved by caspases in the cytoplasm and translocated to the nucleus via perinuclear sites in Huntington's disease patient lymphoblasts. Neurobiol Dis. 2005 Nov;20(2):267-74.
PubMed citation
Sayer JA, Manczak M, Akileswaran L, Reddy PH, Coghlan VM. Interaction of the nuclear matrix protein NAKAP with HypA and huntingtin: implications for nuclear toxicity in Huntington's disease pathogenesis. Neuromolecular Med. 2005;7(4):297-310.
PubMed citation
Schaffar G, Breuer P, Boteva R, Behrends C, Tzvetkov N, Strippel N, Sakahira H, Siegers K, Hayer-Hartl M, Hartl FU. Cellular toxicity of polyglutamine expansion proteins: mechanism of transcription factor deactivation. Mol Cell. 2004 Jul 2;15(1):95-105.
PubMed citation
Schaffar G, Breuer P, Boteva R, Behrends C, Tzvetkov N, Strippel N, Sakahira H, Siegers K, Hayer-Hartl M, Hartl FU. Cellular toxicity of polyglutamine expansion proteins: mechanism of transcription factor deactivation. Mol Cell. 2004 Jul 2;15(1):95-105.
PubMed citation
Schaffar G, Breuer P, Boteva R, Behrends C, Tzvetkov N, Strippel N, Sakahira H, Siegers K, Hayer-Hartl M, Hartl FU. Cellular toxicity of polyglutamine expansion proteins: mechanism of transcription factor deactivation. Mol Cell. 2004 Jul 2;15(1):95-105.
PubMed citation
Schilling B, Gafni J, Torcassi C, Cong X, Row RH, LaFevre-Bernt MA, Cusack MP, Ratovitski T, Hirschhorn R, Ross CA, Gibson BW, Ellerby LM. Huntingtin phosphorylation sites mapped by mass spectrometry. Modulation of cleavage and toxicity. J Biol Chem. 2006 Aug 18;281(33):23686-97. Epub 2006 Jun 16.
PubMed citation
Schwab C, Arai T, Hasegawa M, Yu S, McGeer PL. Colocalization of transactivation-responsive DNA-binding protein 43 and huntingtin in inclusions of Huntington disease. J Neuropathol Exp Neurol. 2008 Dec;67(12):1159-65.
PubMed citation
Seneca S, Fagnart D, Keymolen K, Lissens W, Hasaerts D, Debulpaep S, Desprechins B, Liebaers I, De Meirleir L. Early onset Huntington disease: a neuronal degeneration syndrome. Eur J Pediatr. 2004 Dec;163(12):717-21.
PubMed citation
Seo H, Kim W, Isacson O. Compensatory changes in the ubiquitin-proteasome system, brain-derived neurotrophic factor and mitochondrial complex II/III in YAC72 and R6/2 transgenic mice partially model Huntington's disease patients. Hum Mol Genet. 2008 Oct 15;17(20):3144-53. Epub 2008 Jul 17.
PubMed citation
Seong IS, Ivanova E, Lee JM, Choo YS, Fossale E, Anderson M, Gusella JF, Laramie JM, Myers RH, Lesort M, MacDonald ME. HD CAG repeat implicates a dominant property of huntingtin in mitochondrial energy metabolism. Hum Mol Genet. 2005 Oct 1;14(19):2871-80. Epub 2005 Aug 22.
PubMed citation
Shelbourne PF, Keller-McGandy C, Bi WL, Yoon SR, Dubeau L, Veitch NJ, Vonsattel JP, Wexler NS; US-Venezuela Collaborative Research Group; Arnheim N, Augood SJ. Triplet repeat mutation length gains correlate with cell-type specific vulnerability in Huntington disease brain. Hum Mol Genet. 2007 May 15;16(10):1133-42. Epub 2007 Apr 4.
PubMed citation
Shibata M, Lu T, Furuya T, Degterev A, Mizushima N, Yoshimori T, MacDonald M, Yankner B, Yuan J. Regulation of intracellular accumulation of mutant Huntingtin by Beclin 1. J Biol Chem. 2006 May 19;281(20):14474-85. Epub 2006 Mar 6.
PubMed citation
Shin JY, Fang ZH, Yu ZX, Wang CE, Li SH, Li XJ. Expression of mutant huntingtin in glial cells contributes to neuronal excitotoxicity. J Cell Biol. 2005 Dec 19;171(6):1001-12. Erratum in: J Cell Biol. 2006 Mar 13;172(6):953.
PubMed citation
Singaraja RR, Hadano S, Metzler M, Givan S, Wellington CL, Warby S, Yanai A, Gutekunst CA, Leavitt BR, Yi H, Fichter K, Gan L, McCutcheon K, Chopra V, Michel J, Hersch SM, Ikeda JE, Hayden MR. HIP14, a novel ankyrin domain-containing protein, links huntingtin to intracellular trafficking and endocytosis. Hum Mol Genet. 2002 Nov 1;11(23):2815-28.
PubMed citation
Singaraja RR, Hadano S, Metzler M, Givan S, Wellington CL, Warby S, Yanai A, Gutekunst CA, Leavitt BR, Yi H, Fichter K, Gan L, McCutcheon K, Chopra V, Michel J, Hersch SM, Ikeda JE, Hayden MR. HIP14, a novel ankyrin domain-containing protein, links huntingtin to intracellular trafficking and endocytosis. Hum Mol Genet. 2002 Nov 1;11(23):2815-28.
PubMed citation
Singaraja RR, Hadano S, Metzler M, Givan S, Wellington CL, Warby S, Yanai A, Gutekunst CA, Leavitt BR, Yi H, Fichter K, Gan L, McCutcheon K, Chopra V, Michel J, Hersch SM, Ikeda JE, Hayden MR. HIP14, a novel ankyrin domain-containing protein, links huntingtin to intracellular trafficking and endocytosis. Hum Mol Genet. 2002 Nov 1;11(23):2815-28.
PubMed citation
Singaraja RR, Hadano S, Metzler M, Givan S, Wellington CL, Warby S, Yanai A, Gutekunst CA, Leavitt BR, Yi H, Fichter K, Gan L, McCutcheon K, Chopra V, Michel J, Hersch SM, Ikeda JE, Hayden MR. HIP14, a novel ankyrin domain-containing protein, links huntingtin to intracellular trafficking and endocytosis. Hum Mol Genet. 2002 Nov 1;11(23):2815-28.
PubMed citation
Sipione S, Cattaneo E. Modeling Huntington's disease in cells, flies, and mice. Mol Neurobiol. 2001 Feb;23(1):21-51.
PubMed citation
Sittler A, Wälter S, Wedemeyer N, Hasenbank R, Scherzinger E, Eickhoff H, Bates GP, Lehrach H, Wanker EE. SH3GL3 associates with the Huntingtin exon 1 protein and promotes the formation of polygln-containing protein aggregates. Mol Cell. 1998 Oct;2(4):427-36.
PubMed citation
Sittler A, Wälter S, Wedemeyer N, Hasenbank R, Scherzinger E, Eickhoff H, Bates GP, Lehrach H, Wanker EE. SH3GL3 associates with the Huntingtin exon 1 protein and promotes the formation of polygln-containing protein aggregates. Mol Cell. 1998 Oct;2(4):427-36.
PubMed citation
Sittler A, Wälter S, Wedemeyer N, Hasenbank R, Scherzinger E, Eickhoff H, Bates GP, Lehrach H, Wanker EE. SH3GL3 associates with the Huntingtin exon 1 protein and promotes the formation of polygln-containing protein aggregates. Mol Cell. 1998 Oct;2(4):427-36.
PubMed citation
Solans A, Zambrano A, Rodríguez M, Barrientos A. Cytotoxicity of a mutant huntingtin fragment in yeast involves early alterations in mitochondrial OXPHOS complexes II and III. Hum Mol Genet. 2006 Oct 15;15(20):3063-81. Epub 2006 Sep 12.
PubMed citation
Song C, Zhang Y, Parsons CG, Liu YF. Expression of polyglutamine-expanded huntingtin induces tyrosine phosphorylation of N-methyl-D-aspartate receptors. J Biol Chem. 2003 Aug 29;278(35):33364-9. Epub 2003 Jun 16.
PubMed citation
Steffan JS, Agrawal N, Pallos J, Rockabrand E, Trotman LC, Slepko N, Illes K, Lukacsovich T, Zhu YZ, Cattaneo E, Pandolfi PP, Thompson LM, Marsh JL. SUMO modification of Huntingtin and Huntington's disease pathology. Science. 2004 Apr 2;304(5667):100-4.
PubMed citation
Steffan JS, Kazantsev A, Spasic-Boskovic O, Greenwald M, Zhu YZ, Gohler H, Wanker EE, Bates GP, Housman DE, Thompson LM. The Huntington's disease protein interacts with p53 and CREB-binding protein and represses transcription. Proc Natl Acad Sci U S A. 2000 Jun 6;97(12):6763-8.
PubMed citation
Steffan JS, Kazantsev A, Spasic-Boskovic O, Greenwald M, Zhu YZ, Gohler H, Wanker EE, Bates GP, Housman DE, Thompson LM. The Huntington's disease protein interacts with p53 and CREB-binding protein and represses transcription. Proc Natl Acad Sci U S A. 2000 Jun 6;97(12):6763-8.
PubMed citation
Steffan JS, Kazantsev A, Spasic-Boskovic O, Greenwald M, Zhu YZ, Gohler H, Wanker EE, Bates GP, Housman DE, Thompson LM. The Huntington's disease protein interacts with p53 and CREB-binding protein and represses transcription. Proc Natl Acad Sci U S A. 2000 Jun 6;97(12):6763-8.
PubMed citation
Steffan JS, Kazantsev A, Spasic-Boskovic O, Greenwald M, Zhu YZ, Gohler H, Wanker EE, Bates GP, Housman DE, Thompson LM. The Huntington's disease protein interacts with p53 and CREB-binding protein and represses transcription. Proc Natl Acad Sci U S A. 2000 Jun 6;97(12):6763-8.
PubMed citation
Steffan JS, Kazantsev A, Spasic-Boskovic O, Greenwald M, Zhu YZ, Gohler H, Wanker EE, Bates GP, Housman DE, Thompson LM. The Huntington's disease protein interacts with p53 and CREB-binding protein and represses transcription. Proc Natl Acad Sci U S A. 2000 Jun 6;97(12):6763-8.
PubMed citation
Steffan JS, Kazantsev A, Spasic-Boskovic O, Greenwald M, Zhu YZ, Gohler H, Wanker EE, Bates GP, Housman DE, Thompson LM. The Huntington's disease protein interacts with p53 and CREB-binding protein and represses transcription. Proc Natl Acad Sci U S A. 2000 Jun 6;97(12):6763-8.
PubMed citation
Steffan JS, Kazantsev A, Spasic-Boskovic O, Greenwald M, Zhu YZ, Gohler H, Wanker EE, Bates GP, Housman DE, Thompson LM. The Huntington's disease protein interacts with p53 and CREB-binding protein and represses transcription. Proc Natl Acad Sci U S A. 2000 Jun 6;97(12):6763-8.
PubMed citation
Strehlow AN, Li JZ, Myers RM. Wild-type huntingtin participates in protein trafficking between the Golgi and the extracellular space. Hum Mol Genet. 2007 Feb 15;16(4):391-409. Epub 2006 Dec 22.
PubMed citation
Sun Y, Savanenin A, Reddy PH, Liu YF. Polyglutamine-expanded huntingtin promotes sensitization of N-methyl-D-aspartate receptors via post-synaptic density 95. J Biol Chem. 2001 Jul 6;276(27):24713-8. Epub 2001 Apr 23.
PubMed citation
Sułek-Piatkowska A, Krysa W, Zdzienicka E, Szirkowiec W, Hoffman-Zacharska D, Rajkiewicz M, Fidziańska E, Kowalska G, Zaremba J. Searching for mutation in the JPH3, ATN1 and TBP genes in Polish patients suspected of Huntington's disease and without mutation in the IT15 gene. Neurol Neurochir Pol. 2008 May-Jun;42(3):203-9.
PubMed citation
Swayne LA, Braun JE. Aggregate-centered redistribution of proteins by mutant huntingtin. Biochem Biophys Res Commun. 2007 Mar 2;354(1):39-44. Epub 2006 Dec 26.
PubMed citation
Swayne LA, Chen L, Hameed S, Barr W, Charlesworth E, Colicos MA, Zamponi GW, Braun JE. Crosstalk between huntingtin and syntaxin 1A regulates N-type calcium channels. Mol Cell Neurosci. 2005 Nov;30(3):339-51. Epub 2005 Sep 12.
PubMed citation
Tagawa K, Hoshino M, Okuda T, Ueda H, Hayashi H, Engemann S, Okado H, Ichikawa M, Wanker EE, Okazawa H. Distinct aggregation and cell death patterns among different types of primary neurons induced by mutant huntingtin protein. J Neurochem. 2004 May;89(4):974-87.
PubMed citation
Tang TS, Tu H, Chan EY, Maximov A, Wang Z, Wellington CL, Hayden MR, Bezprozvanny I. Huntingtin and huntingtin-associated protein 1 influence neuronal calcium signaling mediated by inositol-(1,4,5) triphosphate receptor type 1. Neuron. 2003 Jul 17;39(2):227-39.
PubMed citation
Torres-Peraza JF, Giralt A, García-Martínez JM, Pedrosa E, Canals JM, Alberch J. Disruption of striatal glutamatergic transmission induced by mutant huntingtin involves remodeling of both postsynaptic density and NMDA receptor signaling. Neurobiol Dis. 2008 Mar;29(3):409-21. Epub 2007 Oct 23.
PubMed citation
Truant R, Atwal RS, Burtnik A. Nucleocytoplasmic trafficking and transcription effects of huntingtin in Huntington's disease. Prog Neurobiol. 2007 Nov;83(4):211-27. Epub 2007 Jan 22. Review.
PubMed citation
Trushina E, Dyer RB, Badger JD 2nd, Ure D, Eide L, Tran DD, Vrieze BT, Legendre-Guillemin V, McPherson PS, Mandavilli BS, Van Houten B, Zeitlin S, McNiven M, Aebersold R, Hayden M, Parisi JE, Seeberg E, Dragatsis I, Doyle K, Bender A, Chacko C, McMurray CT. Mutant huntingtin impairs axonal trafficking in mammalian neurons in vivo and in vitro. Mol Cell Biol. 2004 Sep;24(18):8195-209.
PubMed citation
Trushina E, Heldebrant MP, Perez-Terzic CM, Bortolon R, Kovtun IV, Badger JD 2nd, Terzic A, Estévez A, Windebank AJ, Dyer RB, Yao J, McMurray CT. Microtubule destabilization and nuclear entry are sequential steps leading to toxicity in Huntington's disease. Proc Natl Acad Sci U S A. 2003 Oct 14;100(21):12171-6. Epub 2003 Oct 3.
PubMed citation
Vacher C, Garcia-Oroz L, Rubinsztein DC. Overexpression of yeast hsp104 reduces polyglutamine aggregation and prolongs survival of a transgenic mouse model of Huntington's disease. Hum Mol Genet. 2005 Nov 15;14(22):3425-33. Epub 2005 Oct 4.
PubMed citation
van Dellen A, Cordery PM, Spires TL, Blakemore C, Hannan AJ. Wheel running from a juvenile age delays onset of specific motor deficits but does not alter protein aggregate density in a mouse model of Huntington's disease. BMC Neurosci. 2008 Apr 1;9:34.
PubMed citation
van Duijn E, Kingma EM, Timman R, Zitman FG, Tibben A, Roos RA, van der Mast RC. Cross-Sectional Study on Prevalences of Psychiatric Disorders in Mutation Carriers of Huntington's Disease Compared With Mutation-Negative First-Degree Relatives. J Clin Psychiatry. 2008 Nov 24; [Epub ahead of print].
PubMed citation
Van Raamsdonk JM, Murphy Z, Selva DM, Hamidizadeh R, Pearson J, Petersén A, Björkqvist M, Muir C, Mackenzie IR, Hammond GL, Vogl AW, Hayden MR, Leavitt BR. Testicular degeneration in Huntington disease. Neurobiol Dis. 2007 Jun;26(3):512-20. Epub 2007 Jan 27.
PubMed citation
Wang CE, Tydlacka S, Orr AL, Yang SH, Graham RK, Hayden MR, Li S, Chan AW, Li XJ. Accumulation of N-terminal mutant huntingtin in mouse and monkey models implicated as a pathogenic mechanism in Huntington's disease. Hum Mol Genet. 2008 Sep 1;17(17):2738-51. Epub 2008 Jun 16.
PubMed citation
Wang CE, Zhou H, McGuire JR, Cerullo V, Lee B, Li SH, Li XJ. Suppression of neuropil aggregates and neurological symptoms by an intracellular antibody implicates the cytoplasmic toxicity of mutant huntingtin. J Cell Biol. 2008 Jun 2;181(5):803-16. Epub 2008 May 26.
PubMed citation
Warby SC, Chan EY, Metzler M, Gan L, Singaraja RR, Crocker SF, Robertson HA, Hayden MR. Huntingtin phosphorylation on serine 421 is significantly reduced in the striatum and by polyglutamine expansion in vivo. Hum Mol Genet. 2005 Jun 1;14(11):1569-77. Epub 2005 Apr 20.
PubMed citation
Warby SC, Doty CN, Graham RK, Carroll JB, Yang YZ, Singaraja RR, Overall CM, Hayden MR. Activated caspase-6 and caspase-6-cleaved fragments of huntingtin specifically colocalize in the nucleus. Hum Mol Genet. 2008 Aug 1;17(15):2390-404. Epub 2008 Apr 29.
PubMed citation
Wellington CL, Ellerby LM, Hackam AS, Margolis RL, Trifiro MA, Singaraja R, McCutcheon K, Salvesen GS, Propp SS, Bromm M, Rowland KJ, Zhang T, Rasper D, Roy S, Thornberry N, Pinsky L, Kakizuka A, Ross CA, Nicholson DW, Bredesen DE, Hayden MR. Caspase cleavage of gene products associated with triplet expansion disorders generates truncated fragments containing the polyglutamine tract. J Biol Chem. 1998 Apr 10;273(15):9158-67.
PubMed citation
Wellington CL, Ellerby LM, Hackam AS, Margolis RL, Trifiro MA, Singaraja R, McCutcheon K, Salvesen GS, Propp SS, Bromm M, Rowland KJ, Zhang T, Rasper D, Roy S, Thornberry N, Pinsky L, Kakizuka A, Ross CA, Nicholson DW, Bredesen DE, Hayden MR. Caspase cleavage of gene products associated with triplet expansion disorders generates truncated fragments containing the polyglutamine tract. J Biol Chem. 1998 Apr 10;273(15):9158-67.
PubMed citation
Wellington CL, Ellerby LM, Hackam AS, Margolis RL, Trifiro MA, Singaraja R, McCutcheon K, Salvesen GS, Propp SS, Bromm M, Rowland KJ, Zhang T, Rasper D, Roy S, Thornberry N, Pinsky L, Kakizuka A, Ross CA, Nicholson DW, Bredesen DE, Hayden MR. Caspase cleavage of gene products associated with triplet expansion disorders generates truncated fragments containing the polyglutamine tract. J Biol Chem. 1998 Apr 10;273(15):9158-67.
PubMed citation
Westhoff B, Chapple JP, van der Spuy J, Höhfeld J, Cheetham ME. HSJ1 is a neuronal shuttling factor for the sorting of chaperone clients to the proteasome. Curr Biol. 2005 Jun 7;15(11):1058-64.
PubMed citation
Wheeler VC, Persichetti F, McNeil SM, Mysore JS, Mysore SS, MacDonald ME, Myers RH, Gusella JF, Wexler NS; US-Venezuela Collaborative Research Group. Factors associated with HD CAG repeat instability in Huntington disease. J Med Genet. 2007 Nov;44(11):695-701. Epub 2007 Jul 27.
PubMed citation
Willingham S, Outeiro TF, DeVit MJ, Lindquist SL, Muchowski PJ. Yeast genes that enhance the toxicity of a mutant huntingtin fragment or alpha-synuclein. Science. 2003 Dec 5;302(5651):1769-72.
PubMed citation
Wu J, Lin F, Qin Z. Sequestration of glyceraldehyde-3-phosphate dehydrogenase to aggregates formed by mutant huntingtin. Acta Biochim Biophys Sin (Shanghai). 2007 Nov;39(11):885-90.
PubMed citation
Yang H, Zhong X, Ballar P, Luo S, Shen Y, Rubinsztein DC, Monteiro MJ, Fang S. Ubiquitin ligase Hrd1 enhances the degradation and suppresses the toxicity of polyglutamine-expanded huntingtin. Exp Cell Res. 2007 Feb 1;313(3):538-50. Epub 2006 Nov 3.
PubMed citation
Yohrling GJ, Farrell LA, Hollenberg AN, Cha JH. Mutant huntingtin increases nuclear corepressor function and enhances ligand-dependent nuclear hormone receptor activation. Mol Cell Neurosci. 2003 May;23(1):28-38.
PubMed citation
Yohrling IV GJ, Jiang GC, DeJohn MM, Robertson DJ, Vrana KE, Cha JH. Inhibition of tryptophan hydroxylase activity and decreased 5-HT1A receptor binding in a mouse model of Huntington's disease. J Neurochem. 2002 Sep;82(6):1416-23.
PubMed citation
Young AB. Huntingtin in health and disease. J Clin Invest. 2003 Feb;111(3):299-302. Review. No abstract available.
PubMed citation
Yu ZX, Li SH, Evans J, Pillarisetti A, Li H, Li XJ. Mutant huntingtin causes context-dependent neurodegeneration in mice with Huntington's disease. J Neurosci. 2003 Mar 15;23(6):2193-202.
PubMed citation
Yu ZX, Li SH, Nguyen HP, Li XJ. Huntingtin inclusions do not deplete polyglutamine-containing transcription factors in HD mice. Hum Mol Genet. 2002 Apr 15;11(8):905-14.
PubMed citation
Zainelli GM, Ross CA, Troncoso JC, Fitzgerald JK, Muma NA. Calmodulin regulates transglutaminase 2 cross-linking of huntingtin. J Neurosci. 2004 Feb 25;24(8):1954-61.
PubMed citation
Zala D, Benchoua A, Brouillet E, Perrin V, Gaillard MC, Zurn AD, Aebischer P, Déglon N. Progressive and selective striatal degeneration in primary neuronal cultures using lentiviral vector coding for a mutant huntingtin fragment. Neurobiol Dis. 2005 Dec;20(3):785-98. Epub 2005 Jul 11.
PubMed citation
Zeron MM, Hansson O, Chen N, Wellington CL, Leavitt BR, Brundin P, Hayden MR, Raymond LA. Increased sensitivity to N-methyl-D-aspartate receptor-mediated excitotoxicity in a mouse model of Huntington's disease. Neuron. 2002 Mar 14;33(6):849-60.
PubMed citation
Zhang Y, Leavitt BR, van Raamsdonk JM, Dragatsis I, Goldowitz D, MacDonald ME, Hayden MR, Friedlander RM. Huntingtin inhibits caspase-3 activation. EMBO J. 2006 Dec 13;25(24):5896-906. Epub 2006 Nov 23.
PubMed citation
Zuchner T, Brundin P. Mutant huntingtin can paradoxically protect neurons from death. Cell Death Differ. 2008 Mar;15(3):435-42. Epub 2007 Nov 2. Review.
PubMed citation
Published: February 6, 2009