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| Contributors | |
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| Preface | |
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| Mitochondrial Structure and Function | |
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| Mitochondrial DNA Structure and Function | |
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| Mammalian Mitochondrial Genomes | |
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| The Human mtDNA | |
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| Structure of the Human mtDNA D-Loop Region | |
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| Mitochondrial DNA Replication | |
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| Initiation of L-Strand DNA Replication | |
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| Alternative Mode of mtDNA Replication | |
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| General Features of Factors Associated with mtDNA Replication | |
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| Regulation of mtDNA Replication | |
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| Mitochondrial Transcription | |
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| Translation of Mitochondrial Transcripts | |
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| Concluding Remarks | |
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| References | |
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| Oxidative Phosphorylation: Structure, Function, and Intermediary Metabolism | |
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| Historical Background | |
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| The Mitochondrial Electron Transport Chain | |
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| Intermediary Metabolism | |
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| Concluding Remarks | |
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| References | |
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| Import of Mitochondrial Proteins | |
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| Introduction | |
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| The Pathways of Mitochondrial Preprotein Import | |
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| Mitochondrial Biogenesis and Human Neurodegenerative Diseases | |
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| References | |
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| Primary Respiratory Chain Disorders | |
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| Mitochondrial Disorders of the Nervous System: Clinical, Biochemical, and Molecular Genetic Features | |
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| Introduction | |
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| Historical Considerations | |
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| Genetics and Pedigree Studies | |
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| Clinical Features | |
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| Important Clinical Questions | |
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| Major Mitochondrial Syndromes | |
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| Biochemical Features | |
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| Diagnostic Approaches | |
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| Treatment of Mitochondrial Disorders | |
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| References | |
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| Secondary Respiratory Chain Disorders | |
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| Friedreich's Ataxia | |
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| Features of Friedreich's Ataxia | |
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| Effect of FRDA Gene Mutations | |
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| Models of FRDA | |
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| FRDA Molecular Mechanisms | |
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| Therapeutic Intervention | |
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| Conclusion | |
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| References | |
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| Wilson Disease | |
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| The Role and Transport of Copper in Health | |
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| Aceruloplasminemia and Menkes' Disease | |
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| Experimental Models of Wilson Disease | |
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| Mitochondrial Dysfunction in Animal Models | |
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| Mitochondrial Dysfunction in Human Disease | |
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| Molecular Basis for the Variation in Phenotype | |
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| Diagnosis | |
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| Cranial Magnetic Resonance Imaging (MRI) and Spectroscopy in Wilson Disease | |
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| Treatment | |
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| Conclusion | |
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| References | |
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| Hereditary Spastic Paraplegia | |
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| Introduction | |
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| Clinical Features | |
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| Genetics | |
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| References | |
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| Cytochrome c Oxidase Deficiency | |
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| Cytochrome c Oxidase Biogenesis | |
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| Nuclear Genes Affecting COX Assembly and Stability | |
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| Nuclear Genes Affecting mtDNA Level and/or Stability | |
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| mtDNA Defects | |
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| References | |
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| Toxin-Induced Mitochondrial Dysfunction | |
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| Toxin-Induced Mitochondrial Dysfunction | |
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| Introduction | |
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| Inhibitors of Mitochondrial Complex I: NADH Ubiquinine Oxidoreductase | |
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| Inhibitors of Mitochondrial Complex II: Succinate Ubiquinol Oxidoreductase | |
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| Inhibitors of Mitochondrial Complex IV: Cytochrome c Oxidase | |
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| Manganese | |
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| 3-Acetylpyridine | |
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| Myopathies and Myotoxic Agents | |
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| Discussion: What Determines the Regional and Cellular Specificity of Mitochondrial Toxins? | |
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| References | |
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| Neurodegenerative Disorders | |
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| Parkinson's Disease | |
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| Introduction | |
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| Mitochondrial Dysfunction in Parkinson's Disease | |
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| Etiology of Parkinson's Disease | |
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| Mitochondrial Dysfunction and the Pathophysiology of Parkinson's Disease | |
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| Concluding Remarks | |
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| References | |
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| Huntington's Disease: The Mystery Unfolds? | |
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| Huntington's Disease | |
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| Epidemiology and Symptomatology | |
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| Neuropathology | |
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| Genetics | |
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| Other Triplet Repeat Diseases | |
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| Intracellular Localization of Normal and Mutant Huntingtin | |
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| Function of Normal and Mutant Huntingtin | |
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| Cell Death in Huntington's Disease: Apoptosis and Authophagy | |
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| Oxidative Stress and Metabolic Dysfunction | |
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| Dopamine Toxicity | |
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| Transgenic Mouse Models of Huntington's Disease | |
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| Concluding Remarks | |
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| References | |
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| Mitochondria in Alzheimer's Disease | |
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| Introduction | |
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| Historical Overview and the Amyloid Cascade Hypothesis | |
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| Metabolic Dysfunction in Alzheimer's Disease has been Reported | |
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| Morphological Studies Demonstrate Mitochondrial Abnormalities in Alzheimer's Disease | |
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| PDHC and KGDHC in Alzheimer's Disease | |
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| Brain Biochemical Studies of Mitochondrial Enzymes in Alzheimer's Disease: Is Cytochrome Oxidase Reduction Characteristic of Alzheimer's Disease? | |
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| Is a Brain Cytochrome Oxidase Deficiency a Robust Feature of Alzheimer's Disease? | |
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| Studies of Cytochrome Oxidase in Non-CNS Tissues in Alzheimer's Disease: Clues to the Origin of the Enzyme Change? | |
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| Cytochrome Oxidase Dysfunction in Alzheimer's Disease: Possible Genetic Component? | |
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| Cytochrome Oxidase Dysfunction in Alzheimer's Disease: Genetic Studies are Still Inconclusive | |
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| Cybrid Data Suggest mtDNA Contributes to Alzheimer's Disease Cytochrome Oxidase Dysfunction | |
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| Unresolved Issues in Alzheimer's Disease Cybrid Studies: Where is the mtDNA "Mutation?" | |
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| Could a Cytochrome Oxidase Defect Cause Alzheimer's Disease? | |
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| Concluding Remarks | |
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| References | |
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| Contributions of Mitochondrial Alterations, Resulting from Bad Genes and a Hostile Environment, to the Pathogenesis of Alzheimer's Disease | |
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| Overview of Neurodegenerative Cascades in Alzheimer's Disease | |
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| Mitochondrial Alterations in Alzheimer's Disease Patients and Experimental Models | |
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| Genetic Factors and Mitochondrial Alterations in Alzheimer's Disease | |
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| Environmental Factors and Mitochondrial Alterations in Alzheimer's Disease | |
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| Conclusions | |
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| References | |
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| Mitochondria and Amyotrophic Lateral Sclerosis | |
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| Introduction | |
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| Clinical Features | |
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| Pathogenic Hypotheses | |
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| Transgenic Mouse Models of ALS | |
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| Conclusion | |
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| References | |
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| Models of Mitochondrial Disease | |
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| Models of Mitochondrial Disease | |
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| Introduction | |
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| Classification of mtDNA Defects Causing Respiratory Chain Disease | |
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| Cell Models Employed to Study mtDNA Defects | |
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| Cell Models of Respiratory Chain Disease Associated with Specific mtDNA Defects | |
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| Classification of Nuclear DNA Defects Causing Respiratory Chain Disease | |
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| Cell Models of Respiratory Chain Disease Associated with Nuclear DNA Defects | |
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| Application of Cell Models for the Development of Therapeutic Strategies in mtDNA Disease | |
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| Animal Models of Respiratory Chain Disease | |
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| Other Models of Respiratory Chain Disease | |
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| Conclusions | |
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| References | |
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| Defects of [beta]-Oxidation Including Carnitine Deficiency | |
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| Defects of [beta]-Oxidation Including Carnitine Deficiency | |
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| Introduction | |
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| Background Biochemistry | |
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| Inherited Disorders of Mitochondrial [beta]-Oxidation | |
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| Conclusions | |
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| References | |
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| Mitochondrial Involvement in Aging | |
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| The Mitochondrial Theory of Aging: Involvement of Mitochondrial DNA Damage and Repair | |
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| Mitochondria: The Biological Clock? | |
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| Oxidative Damage to Mitochondria | |
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| Accumulation of Oxidative Damage to mtDNA | |
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| DNA Repair in Mammalian Mitochondria | |
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| Changes in mtDNA Repair with Age | |
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| Conclusions | |
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| References | |
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| Index | |
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| Contents of Recent Volumes | |