Bindoff L A, Birch-Machin M A, Cartlidge N E, Parker W D, Turnbull D M
Division of Clinical Neuroscience, Medical School, University of Newcastle upon Tyne, U.K.
J Neurol Sci. 1991 Aug;104(2):203-8. doi: 10.1016/0022-510x(91)90311-t.
Parkinson's disease is one of the commonest neurodegenerative disorders in Western society. Although the neuropathological changes have been well documented, the underlying biochemical defect is unknown. Toxins may play a part in the aetiology of this disorder. It has been shown that 1-methyl-4-phenyl-1,2,3,6-tetrahydropyridine (MPTP) produces a Parkinson-like syndrome in both man and primates and 1-methyl-4-phenylpyridine (MPP+), a metabolite of MPTP, inhibits NADH-ubiquinone oxidoreductase (complex I) of the mitochondrial respiratory chain. We studied mitochondrial respiratory chain function in skeletal muscle from patients with Parkinson's disease because, like brain, it has a high dependence on oxidative metabolism. Our results show low activity in all complexes studied (I, II and IV). The implications of these findings are discussed in relation to the aetiology of Parkinson's disease.
帕金森病是西方社会最常见的神经退行性疾病之一。尽管神经病理学变化已有充分记载，但其潜在的生化缺陷尚不清楚。毒素可能在这种疾病的病因中起作用。研究表明，1-甲基-4-苯基-1,2,3,6-四氢吡啶（MPTP）在人类和灵长类动物中都会产生帕金森样综合征，而MPTP的代谢产物1-甲基-4-苯基吡啶（MPP+）会抑制线粒体呼吸链的NADH-泛醌氧化还原酶（复合体I）。我们研究了帕金森病患者骨骼肌中的线粒体呼吸链功能，因为与大脑一样，骨骼肌高度依赖氧化代谢。我们的结果显示，所研究的所有复合体（I、II和IV）活性均较低。本文结合帕金森病的病因对这些发现的意义进行了讨论。
