Front Cover -- Molecular Neurology -- Copyright Page -- Contents -- Contributor's List -- Preface -- Section I: Principles of Molecular Neurology -- Chapter 1: Genetics as a Tool in Neurology -- I. Introduction -- II. Structure and Function of Genes and Chromosomes -- III. Genetic Medicine -- IV. The Neurogenetic Evaluation -- V. Identification of Human Disease Genes -- VI. Methods for Human Molecular Genetic Analysis -- VII. Treatment of Genetic Diseases -- References -- Chapter 2: Neurology and Genomic Medicine -- I. Introduction -- II. Basic Concepts -- III. The Human Genome Project (HGP) and Haplotype Mapping (HapMap) Project -- IV. Family History -- V. Genetic Mechanisms -- VI. Pharmacogenetics -- VII. Gene-Gene and Gene-Environment Interactions -- VIII. Comparative Genomic Hybridization (CGH) -- IX. Mitochondria and the Mitochondrial Genome (mtDNA) -- X. Summary -- References -- Chapter 3: Mitochondrial Function and Dysfunction in the Nervous System -- I. Introduction -- II. Structure and Functions of Mitochondria -- III. Mitochondria in Mechanisms of Neuronal Cell Death and Neurological Disease -- IV. Roles of Mitochondrial Dysfunction in Common Neurodegenerative Diseases -- V. Conclusions -- References -- Cahpter 4: Neuronal Channels and Receptors -- I. Introduction -- II. Nomenclature -- III. Structure and Function -- IV. Physiological Roles -- V. Neurological Disorders Caused by Channelopathies -- References -- Chapter 5: Protein Misfolding, Chaperone Networks, and the Heat Shock Response in the Nervous System -- I. Introduction -- II. Role of Molecular Chaperones in Protein Folding Quality Control -- III. Regulation of Chaperone Expression: The Heat Shock Response -- IV. Role of Molecular Chaperones in Neurodegenerative Diseases -- V. Chaperone Hypotheses -- VI. Therapeutic Avenues -- References.

Chapter 6: Metabolic Biopsy of the Brain -- I. Phosphorus Magnetic Resonance Spectroscopy -- II. The Phosphocreatine Shuttle Hypothesis -- III. Magnetization Transfer Measurements of ATP and Phosphocreatine Synthesis -- IV. Hydrogen (Proton) Spectroscopy -- V. Carbon Spectroscopy -- VI. MR Spectroscopic Measurements of Cerebral Lactate -- VII. The Astrocyte-Neuron Lactate Shuttle Hypothesis -- VIII. Cerebral Ammonia Metabolism -- IX. Summary -- References -- Chapter 7: Gene Therapy Approaches in Neurology -- I. Why Use Gene Transfer in the Development of Novel Therapies? -- II. Gene Transfer Strategies -- III. Development of Neurological Gene Therapy -- IV. Conclusions-Future Developments -- References -- Chapter 8: Programmed Cell Death and Its Role in Neurological Disease -- I. Introduction: Neurologists and Cell Death -- II. Cell Death: History and Classification -- III. Current Status of Programmed Cell Death Studies -- IV. Trophic Factors and the Concept of Cellular Dependence -- V. Apoptosis Induced by Unfolded, Misfolded, or Alternatively Folded Proteins -- VI. Does Programmed Cell Death Play a Role in Neurodegeneration? -- VII. Are Programmed Cell Death Pathways Appropriate Therapeutic Targets in Neurodegeneration? -- VIII. Death and Resurrection: The Neural Stem Cell Response to Neurodegeneration -- Acknowledgments -- References -- Section II: Disorders of Development -- Chapter 9: Developmental Neurology: A Molecular Perspective -- References -- Chapter 10: Metabolic Diseases of the Nervous System Development -- I. Glucose Transporter Type 1 Deficiency -- II. Menkes Disease -- III. Segawa Disease (Dopa-Responsive Dystonia) -- IV. Disorders of Pyruvate Metabolism -- V. Glycosylation Disorders -- VI. Organic Acidurias -- VII. Urea Cycle Disorders -- VIII. Galactosemia -- IX. Phenylketonuria -- X. Lesch-Nyhan Disease.

XI. Pantothenate Kinase Deficiency -- XII. Smith-Lemli-Opitz Syndrome -- References -- Chapter 11: Genetic Disorders of Neuromuscular Brain Disease -- I. The Motor Unit during Development -- II. Structures and Function of the Neuromuscular System -- III. Diseases of Developing Nerve, the Neuromuscular Junction and Muscle -- References -- Section III: Stroke and Trauma -- Chapter 12: Molecular Mechanisms of Ischemic -- I. Introduction -- II. Hypoxia/Ischemia -- III. Excitotoxicity -- IV. Free Radicals -- V. Inflammation -- VI. Growth Factors -- VII. Gene Expression in Cerebral Ischemia -- VIII. Apoptosis -- IX. Summary -- References -- Chapter 13: Hemorrhagic Brain Disease -- I. Introduction -- II. Angiogenesis and Vasculogenesis -- III. Mendelian Forms of Hemorrhagic Brain Disease -- IV. Primary Hemorrhagic Brain Diseases (Vascular Lesions) -- V. Secondary Hemorrhagic Brain Diseases -- VI. Future Research -- References -- Chapter 14: The Dawn of Molecular and Cellular Therapies for Traumatic Spinal Cord Injury -- I. The Current Clinical Picture -- II. Surmounting Barriers to Axon Regeneration -- III. Cellular Therapies for SCI -- IV. Molecular Adaptations after SCI -- V. Conclusions -- Acknowledgments -- References -- Section IV: Degenerative Diseases -- Chapter 15: Parkinson Disease: Molecular Insights -- I. Introduction -- II. History -- III. Diagnosis -- IV. Pathology -- V. Etiology: Genetics and Environment -- VI. Genes Implicated in PD -- VII. Models of Pathogenesis -- VIII. Therapy -- IX. Summary -- References -- Chapter 16: The Molecular Basis of Alzheimer's Disease -- I. Clinical Features of Alzheimer's Disease -- II. Neuropathology of Alzheimer's Disease -- III. Amyloid Biology and the Genetics of Early-Onset Alzheimer's Disease -- IV. Alzheimer's Disease-A Progressive Neuropathologic Syndrome -- V. Tau Biology in Alzheimer's Disease.

VI. Genetics of Late-Onset Alzheimer's Disease -- VII. Animal Models of Alzheimer's Disease -- VIII. What Makes the Neurons Die? -- IX. Abnormal Protein Conformation-A Unifying Factor in Neurodegeneration? -- X. Emerging Diagnostic Tools -- XI. Disease-Modifying Strategies of Tomorrow -- XII. Summary and Conclusions -- References -- Chapter 17: Polyglutamine Disorders Including Huntington's Disease -- I. Introduction -- II. Clinical and Genetic Features -- III. Protein Misfolding and Failures in Protein Quality Control -- IV. Transcriptional Dysregulation -- V. Mitochondrial Dysfunction -- VI. Excitotoxicity and Calcium Homeostasis -- VII. Axonal Transport Defects -- VIII. Neuronal Dysfunctions versus Neuronal Cell Death -- IX. Cell Autonomous versus Non-cell Autonomous Effects -- X. Conclusion -- References -- Chapter 18: Friedreich's Ataxia and Related DNA Loss-of-Function Disorders -- I. Summary -- II. Epidemiology -- III. Pathology of Friedreich Ataxia -- IV. Clinical Phenotype of Friedreich Ataxia -- V. Ancillary Tests -- VI. Frataxin Gene Structure and Expression -- VII. DNA Mutations -- VIII. Frataxin Function -- IX. Animal Models -- X. Pathogenesis -- XI. Treatment: Directions and Perspectives -- XII. Related Loss-of-Function Disorders -- References -- Chapter 19: DYT1, An Inherited Dystonia -- I. Early-Onset Primary Dystonia and Identifying DYT1 -- II. Gene and Protein Properties -- III. Neuropathology -- IV. Cellular and Animal Models of Disease -- V. Invertebrates -- VI. Mouse -- VII. DYT1 Role in Focal Dystonia -- VIII. DYT1 Phenotype and Endophenotype -- IX. Future Directions -- References -- Chapter 20: Motor Neuron Disease: Amyotrophic Lateral Sclerosis -- I. ALS Background -- II. Clinical Manifestations of ALS -- III. Animal Models of Motor Neuron Diseases -- IV. Molecular Hypotheses in ALS -- V. Axonal Pathology.

VI. Neuroinflammation -- VII. Cell Autonomy in ALS-Contributions from Nonneuronal Cells Pathophysiology -- VIII. Regional Differences in ALS and SOD1 Pathophysiology -- IX. Targeting Therapies to Molecular Pathways -- X. Targeting ALS Subgroups Using RNAi and Antisense Technologies -- XI. Predictive Value of Preclinical Models -- References -- Chapter 21: Genetic Disorders of the Autonomic Nervous System -- I. Introduction -- II. Developmental Abnormalities of the Autonomic Nervous System -- III. Functional Abnormalities of the Autonomic Nervous System -- IV. Future Directions -- References -- Chapter 22: Multiple Sclerosis as a Neurodegenerative Disease -- I. Focal Distribution of Sodium Channels in Myelinated Axons -- II. Demyelination in Multiple Sclerosis -- III. Axonal Degeneration in Multiple Sclerosis -- IV. Sodium Channels and Axonal Injury -- V. Energetics, Ionic Homeostasis, and Axonal Injury -- VI. Molecular Identity of Axonal Sodium Channels -- VII. Sodium Channels and Recovery of Conduction in Demyelinated Axons -- VIII. Na 1.2 and Na 1.6 in Normal and Dysmyelinated Axons -- IX. Axonal Sodium Channels in Demyelinated Axons: Lessons from EAE -- X. Axonal Sodium Channels in Injured Axons: EAE -- XI. Axonal Sodium Channels in MS -- XII. Nav1.2 Channels in Demyelinated Axons: Functional Role -- XIII. Nav1.6 Channels in Demyelinated Axons: Functional Role -- XIV. Sodium Channels in Microglia and Macrophages -- XV. From Neurodegeneration to Neuroprotection? -- Acknowledgments -- References -- Section V: Disorders of Excitation and Transmission -- Chapter 23: Acquired Epilepsy: Cellular and Molecular Mechanisms -- I. Introduction -- II. Temporal Lobe Epilepsy -- III. Post-Traumatic and Post-Stroke Epilepsy -- VI. Rasmussen's Syndrome -- V. Post-Infectious Epilepsy -- VI. Epilepsy Caused by Neoplasms and Other Mass Lesions.

VII. Glia, Brain Microenvironment, and Epilepsy.