Contents:

Preface. 1. Metals in Brain, Metal Transport, Storage and Homeostasis. 1.1 Introduction the importance of metal ions in brain function. 1.2 Metal ion transport and storage iron. 1.3 Metal ion transport and storage copper. 1.4 Metal ion transport and storage zinc. 1.5 Metal ion transport and storage other metals. 1.6 Metals and their homeostasis (with particular reference to iron and copper). References. 2. Oxidative Stress and Redox-Active Metal Ions. 2.1 Introduction the oxygen paradox. 2.2 Reactive oxygen species (ROS). 2.3 Reactive nitrogen species (RNS). 2.4 The targets of ROS and RNS. 2.5 Cytoprotection against oxidative damage. 2.6 Mitochondria, free radicals and signaling. 2.7 Cyclin-dependent kinases. 2.8 Apoptosis programmed cell death. 2.9 ROS, RNS and signal transduction. 2.10 Molecules involved in the inflammatory pathway. References. 3. Parkinsons Disease. 3.1 Proteins involved in Parkinsons disease. 3.2 Metal involvement in Parkinsons disease. 3.3 Risk factors for Parkinsons disease. 3.4 Mitochondrial dysfunction. 3.5 Role of dopamine in Parkinsons disease. References. 4 Alzheimers Disease. 4.1 Proteins involved in Alzheimers disease. 4.2 Metal involvement in Alzheimers disease. 4.3 Genetic and risk factors. 4.4 Mitochondrial function in Alzheimers disease. 4.5 Oxidative stress. 4.6 Role of acetyl choline in Alzheimers disease. References. 5. Huntingtons Disease and Polyglutamine Expansion Neurodegenerative Diseases. 5.1 Introduction an overview of trinucleotide expansion diseases. 5.2 PolyQ diseases. 5.3 Structural models of polyQ protein aggregation. 5.4 Mechanisms of cell death in polyQ diseases. 5.5.Huntingtons disease. 5.6 Other polyQ disease proteins. References. 6 Friedreichs Ataxia and Diseases Associated with Expansion of Non-Coding Triplets. 6.1 Incidence and patholophysiology of Friedreichs ataxia. 6.2 Molecular basis of the disease triplet repeat expansions. 6.3 Molecular basis of the disease frataxin and its role in iron metabolism. 6.4 Other diseases associated with expansion of non-coding triplets. References. 7 Creutzfeldt-Jakob and Other Prion Diseases. 7.1 Introduction. 7.2 A brief history of prion diseases. 7.3 The prion of protein-only hypothesis and conformation-based prion inheritance. 7.4 Mechanisms of conformation-based prion transmission. 7.5 Pathways of prion pathogenesis. 7.6 Prion-metal interactions. References. 8 Amyotrophic Lateral Sclerosis. 8.1 Introduction. 8.2 Major genes involved in ALS. 8.3 Superoxide dismutase and ALS. 8.4 Contributors to disease mechanisms in ALS. 8.5 Other pathways that may cause damage to motor neurons. 8.6 Conclusions. References. 9 Other Neurological Diseases. 9.1 Introduction. 9.2 Aceruloplasminaemia. 9.3 Wilsons and Menkes diseases. 9.4 Hallervorden-Spatz syndrome. 9.5 Neuroferritinopathy. 9.6 Multiple sclerosis. 9.7 HIV-associated dementia. References. 10 Therapeutic Strategies to Combat the Onset and Progression of Neurodegenerative Diseases. 10.1 Parkinsons disease. 10.2 Alzheimers disease. 10.3 Huntingtons disease and other polyQ disorders. 10.4 Friedreichs ataxia and other diseases linked to non-coding triplet repeats. 10.5 Prion diseases. 10.6 Amyotrophic lateral sclerosis, ALS. 10.7 Aceruloplasminaemia. 10.8 Menkes disease. 10.9 Wilsons disease. 10.10 Multiple sclerosis. 10.11 Future direction for therapeutic agents. References. 11 Animal Models of the Various Neurodegenerative Diseases. 11.1 Parkinsons disease animal models. 11.2 Alzheimers disease. 11.3 Huntingtons disease and polyQ. 11.4 Friedreichs ataxia. 11.5 Prion diseases. 11.6 Amyotrophic lateral sclerosis, ALS. 11.7 Aceruloplasminaemia. 11.8 Menkes disease. 11.9 Wilsons disease. 11.10 Multiple sclerosis. References. 12 Concluding Remarks. 12.1 Therapeutic strategies. References. Index.