Parker W D, Boyson S J, Parks J K
Department of Neurology, University of Colorado School of Medicine, Denver.
Ann Neurol. 1989 Dec;26(6):719-23. doi: 10.1002/ana.410260606.
Idiopathic Parkinson's disease may have a low-level familial association but does not follow mendelian patterns of inheritance. Since inheritance of some components of the electron transport chain is nonmendelian and since inhibition of the electron transport chain with the toxin 1-methyl-4-phenyl-1,2,3,6-tetrahydropyridine models Parkinson's disease in humans and animals, we evaluated catalytic activities of the electron transport chain in platelet mitochondria purified from patients with idiopathic Parkinson's disease. All 10 patients studied had significant reductions of complex I (NADH:ubiquinone oxidoreductase) activity. Succinate:cytochrome c oxidoreductase activity was less strikingly reduced. We hypothesize that the complex I abnormality may have an etiological role in the pathogenesis of Parkinson's disease and that this defect may be derived via the mitochondrial genome.
特发性帕金森病可能存在低度家族关联性，但并不遵循孟德尔遗传模式。由于电子传递链某些成分的遗传是非孟德尔式的，且用毒素1-甲基-4-苯基-1,2,3,6-四氢吡啶抑制电子传递链可在人和动物中模拟帕金森病，我们评估了从特发性帕金森病患者中纯化的血小板线粒体中电子传递链的催化活性。所研究的10例患者的复合体I（NADH：泛醌氧化还原酶）活性均显著降低。琥珀酸：细胞色素c氧化还原酶活性的降低则不那么明显。我们推测复合体I异常可能在帕金森病发病机制中具有病因学作用，且这种缺陷可能通过线粒体基因组遗传。
