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Contents HISTORICAL DEVELOPMENT AND PERSPECTIVES OF THE SERIES iii PREFACE TO VOLUME 1 v CONTRIBUTORS TO VOLUME 1 TITLES OF VOLUMES 1-44 IN THE METAL IONS IN BIOLOGICAL SYSTEMS SERIES CONTENTS OF FUTURE VOLUMES IN THE METAL IONS IN LIFE SCIENCES SERIES Chapter 1 THE ROLE OF METAL IONS IN NEUROLOGY. AN INTRODUCTION 1 Dorothea Strozyk and Ashley I. Bush Text Acknowledgments Abbreviations References Chapter 2 PROTEIN FOLDING, MISFOLDING, AND DISEASE Jennifer C. Lee, Judy E. Kim, Ekaterina V. Pletneva, Jasmin Faraone-Mennella, Harry B. Gray, and Jay R. Winkler 1. Introduction 2. Experimental Methods 3. The Denatured State 4. Protein Folding Dynamics 5. ?-Synucleic and Parkinson?s Disease 6. Conclusions and Outlook Acknowledgments Abbreviations and Definitions References Chapter 3 METAL ION BINDING PROPERTIES OF PROTEINS RELATED TO NEURODEGENERATION Henryk Kozlowski, Marek Luczkowski, Daniela Valensin, and Gianni Valensin 1. Introduction 2. Cu2+ Interactions with Mammalian Prion Proteins and Their Fragments 3. Interactions of Metal Ions with the Amyloid Precursor Protein and Its Fragments 4. Concluding Remarks Acknowledgments Abbreviations References Chapter 4 METALLIC PRIONS: MINING THE CORE OF TRANSMISSIBLE SPONGIFORM ENCEPHALOPATHIES David R. Brown 1. Introduction 2. Historical Connections between Copper and Transmissible Spongiform 3. Copper Binding to Prion Protein 4. Copper Coordination by Prion Protein 5. Copper Uptake and Prion Protein Internalisation 6. Prion Protein as an Antioxidant 7. Manganese Binding 8. Transmissible Spongiform Encephalopathies and Metals 9. Conclusions Abbreviations References Chapter 5 THE ROLE OF METAL IONS IN THE AMYLOID PRECURSOR PROTEIN AND IN ALZHEIMER?S DISEASE Thomas A. Bayer and Gerd Multhaup 1. Introduction 2. Amyloid Precursor Protein and Brain Copper Homeostasis 3. Amyloid Precursor Protein and Cu,Zn-Superoxide Dismutase-1 4. General Conclusions Abbreviations References Chapter 6 THE ROLE OF IRON IN THE PATHOGENESIS OF PARKINSON'S DISEASE Manfred Gerlach, Kay Double, Mario E. G¿tz, Moussa B. H. Youdim, and Peter Riederer 1. Introduction 2. Iron in the Ethiology of Parkinson?s Disease 3. Sources of Increased Iron in Parkinson?s Disease 4. Consequences of Iron Overload in Parkinson?s Disease 5. General Conclusions Acknowledgments Abbreviations and Definitions References Chapter 7 IN VIVO ASSESSMENT OF IRON IN HUNTINGTON'S DISEASE AND OTHER AGE-RELATED NEURODEGENERATIVE BRAIN DISEASES George Bartzokis, Po H. Lu, Todd A. Tishler, and Susan Perlman 1. Introduction. Puzzling Changes in Cell Numbers in Huntington?s Disease Brain 2. Human Brain Development and Disease Phenotypes 3. Oligodendrocytes and Iron in Brain Development and Degeneration 4. Transition Metal Metabolism and Proteinopathies 5. In Vivo Measurement of Brain Iron 6. Novel Treatment Considerations 7. Conclusions Acknowledgments Abbreviations References Chapter 8 COPPER-ZINC SUPEROXIDE DISMUTASE AND FAMILIAL AMYOTROPHIC LATERAL SCLEROSIS Lisa J. Whitson and P. John Hart 1. Introduction 2. Molecular Mechanisms of fALS SOD1 Pathogenesis 3. Structural Features of Human SOD1 4. "Wild Type-like" fALS Mutants 5. "Metal-Binding Region" fALS Mutants 6. Monomeric SOD1 and Pathogenesis 7. Conclusions Acknowledgments Abbreviations References Chapter 9 THE MALFUNCTIONING OF COPPER TRANSPORT IN WILSON AND MENKES DISEASES Bibudhendra Sarkar 1. Introduction 2. Clinical and Biochemical Features of Copper Transport Disorders 3. Genes Identified in Copper-Transport Disorders 4. Structure and Function of Copper-Transporting ATPases 5. Treatment of Copper-Transport Disorders 6. Conclusions Acknowledgment Abbreviations References Chapter 10 IRON AND ITS ROLE IN NEURODEGENERATIVE DISEASES Roberta J. Ward and Robert R. Crichton 1. Introduction 2. The Inorganic Chemistry of Iron and Its Role in Human Biology 3. Iron Metabolism 4. The Role of the "Labile Iron Pool" in Free Radical Production 5. The Importance of Iron in Brain 6. The Involvement of Iron in Neurodegenerative Diseases 7. Experimental Approaches to Brain Iron Loading 8. Conclusions Acknowledgments Abbreviations References Chapter 11 THE CHEMICAL INTERPLAY BETWEEN CATECHOLAMINES AND METAL IONS IN NEUROLOGICAL DISEASES Wolfgang Linert, Guy N. L. Jameson, Reginald F. Jameson, and Kurt A. Jellinger 1. General Introduction 2. Neurodegenerative Diseases 3. Relevant In Vitro Chemistry 4. Iron and Parkinson's Disease 5. Relevant Manganese Chemistry 6. Manganese and Manganosis 7. Other Metal Ions and Catecholamines 8. Summary of the Effects of Metal Ions on Autoxidation of Dopamine 9. Conclusions Acknowledgment Abbreviations and Definitions References Chapter 12 ZINC METALLONEUROCHEMISTRY: PHYSIOLOGY, PATHOLOGY, AND PROBES Christopher J. Chang and Stephen J. Lippard 1. Introduction 2. Zinc in the Brain 3. Zinc Sensing for Neuroscience Applications 4. Biomolecule Fluorescent Probes for Zinc CONTENTS xiii 5. Small-Molecule Fluorescent Probes for Zinc 6. Concluding Remarks and Future Directions Acknowledgment Abbreviations References Chapter 13 THE ROLE OF ALUMINUM IN NEUROTOXIC AND NEURODEGENERATIVE PROCESSES Tam s Kiss, Krisztina Gajda-Schrantz, and Paolo Zatta 1. Introduction 2. Chemical Forms of Aluminum in Biological Systems 3. Aluminum Loading in Humans 4. Toxicology of Aluminum in Animals and Humans 5. Aluminum and Alzheimer's Disease 6. General Conclusions Acknowledgments Abbreviations References Chapter 14 NEUROTOXICITY OF CADMIUM, LEAD, AND MERCURY Hana R. Pohl, Henry G. Abadin, and John F. Risher 1. Introduction 2. Cadmium 3. Lead 4. Mercury 5. Conclusions Abbreviations and Definitions References Chapter 15 NEURODEGENERATIVE DISEASES AND METAL IONS. A CONCLUDING OVERVIEW Dorothea Strozyk and Ashley I. Bush 1. Introduction 2. The Medicinal Chemistry of Metal-Centered Brain Disorders 3. Other Neurological Disorders that May Involve Metals 4. Conclusion and Outlook Acknowledgments Abbreviations and Definitions References SUBJECT INDEX
Library of Congress Subject Headings for this publication:
Nervous system -- Degeneration.
Nervous system -- Diseases.
Metal ions -- Health aspects.
Neurodegenerative Diseases -- chemically induced.
Metals -- adverse effects.
Metals -- metabolism.
Prion Diseases -- chemically induced.
Protein Folding.