Mitochondrial Disorders in Neurology

Mitochondrial Disorders in Neurology provides an overview of mitochondrial diseases. This book discusses the effects of mitochondrial dysfunction based on the relevant biochemistry and molecular genetics. The abnormal muscle and mitochondrial morphology in a variety of clinical presentations from isolated ophthalmoplegia to severe encephalopathy are also elaborated. This text likewise deliberates Leber's hereditary optic neuropathy, neurodegenerative disorders, and respiratory chain defects. Other topics covered include mitochondrial DNA and the genetics of mitochondrial disease; cytochrome oxidase deficiency; use of tissue culture in the diagnosis of mitochondrial disease; and advances in mitochondrial genetics. This publication is a good source for clinicians and students concerned with the defective mitochondrial function.

List of ContributorsPreface1 The Structural Organization of the Mitochondrial Respiratory Chain2 Mitochondrial DNA and the Genetics of Mitochondrial Disease3 Mitochondrial Myopathies: Clinical Features, Investigation, Treatment and Genetic Counseling4 Biochemical and Molecular Features of Deficiencies of Complexes I, II and III5 Cytochrome Oxidase Deficiency: Progress and Problems6 Mitochondrial Encephalomyopathies: Lumping, Splitting and Melding7 Intermediary Metabolism8 Human Defects of ß-oxidation: Clinical and Molecular Aspects9 The Use of Tissue Culture in the Diagnosis of Mitochondrial Disease10 Leber's Hereditary Optic Neuropathy11 Recent Advances in Mitochondrial Genetics12 Mitochondrial Dysfunction in Neurodegenerative Disorders and AgingIndex