Mitochondrial dysfunction in progressive supranuclear palsy.
作者： David S Albers , M Flint Beal
DOI: 10.1016/S0197-0186(01)00126-7
关键词:
摘要: A progressive impairment of mitochondrial function has been suggested to play a critical role in the pathogenesis several neurodegenerative diseases, including Parkinson's disease, Alzheimer's disease and Huntington's disease. Mitochondrial dysfunction can lead number deleterious consequences impaired calcium buffering, generation free radicals, activation permeability transition pore secondary excitotoxicity. Progressive supranuclear palsy (PSP) is rare neurological disorder characterized by appearance gaze extrapyramidal symptoms [Arch. Neurol. 10 (1964) 333]. Although etiological basis PSP unknown, compelling evidence from spectroscopy studies patients, biochemical post-mortem brain tissue cybrids emerged that supports contributory bio-energetic defects PSP.
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