Mitochondrial Disorders in the Nervous System
Mitochondrial diseases (encephalomyopathies) have traditionally been ascribed to defects of the respiratory chain, which has helped researchers explain their genetic and clinical complexity. However, other mitochondrial functions are greatly important for the nervous system, including protein import...
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| Veröffentlicht in: | Annual review of neuroscience 2008-01, Vol.31 (1), p.91-123 |
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| container_title | Annual review of neuroscience |
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| creator | DIMAURO, Salvatore SCHON, Eric A |
| description | Mitochondrial diseases (encephalomyopathies) have traditionally been ascribed to defects of the respiratory chain, which has helped researchers explain their genetic and clinical complexity. However, other mitochondrial functions are greatly important for the nervous system, including protein importation, organellar dynamics, and programmed cell death. Defects in genes controlling these functions are attracting increasing attention as causes not only of neurological (and psychiatric) diseases but also of age-related neurodegenerative disorders. After discussing some pathogenic conundrums regarding the neurological manifestations of the respiratory chain defects, we review altered mitochondrial dynamics in the etiology of specific neurological diseases and in the physiopathology of more common neurodegenerative disorders. |
| doi_str_mv | 10.1146/annurev.neuro.30.051606.094302 |
| format | Article |
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| ispartof | Annual review of neuroscience, 2008-01, Vol.31 (1), p.91-123 |
| issn | 0147-006X 1545-4126 |
| language | eng |
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| source | Annual Reviews Complete A-Z List; MEDLINE |
| subjects | Animals Biological and medical sciences Brain Brain Diseases, Metabolic - genetics Brain Diseases, Metabolic - metabolism Brain Diseases, Metabolic - physiopathology Disease Errors of metabolism Genes Genetic Predisposition to Disease - genetics Humans Medical sciences Metabolic diseases Miscellaneous hereditary metabolic disorders Mitochondria - metabolism Mitochondrial Diseases - genetics Mitochondrial Diseases - metabolism Mitochondrial Diseases - physiopathology Mutation - genetics Nervous system Neurodegenerative Diseases - genetics Neurodegenerative Diseases - metabolism Neurodegenerative Diseases - physiopathology Neurons Neurons - metabolism Oxidative Stress - genetics |
| title | Mitochondrial Disorders in the Nervous System |
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