Schapira A H, Cooper J M, Dexter D, Clark J B, Jenner P, Marsden C D
Department of Neurological Science, Royal Free Hospital School of Medicine, London, England.
J Neurochem. 1990 Mar;54(3):823-7. doi: 10.1111/j.1471-4159.1990.tb02325.x.
The structure and function of mitochondrial respiratory-chain enzyme proteins were studied postmortem in the substantia nigra of nine patients with Parkinson's disease and nine matched controls. Total protein and mitochondrial mass were similar in the two groups. NADH-ubiquinone reductase (Complex I) and NADH cytochrome c reductase activities were significantly reduced, whereas succinate cytochrome c reductase activity was normal. These results indicated a specific defect of Complex I activity in the substantia nigra of patients with Parkinson's disease. This biochemical defect is the same as that produced in animal models of parkinsonism by 1-methyl-4-phenyl-1,2,3,6-tetrahydropyridine (MPTP) and adds further support to the proposition that Parkinson's disease may be due to an environmental toxin with action(s) similar to those of MPTP.
对9例帕金森病患者和9例匹配的对照者的黑质进行尸检，研究线粒体呼吸链酶蛋白的结构和功能。两组的总蛋白和线粒体质量相似。NADH-泛醌还原酶（复合体I）和NADH细胞色素c还原酶活性显著降低，而琥珀酸细胞色素c还原酶活性正常。这些结果表明帕金森病患者黑质中复合体I活性存在特异性缺陷。这种生化缺陷与1-甲基-4-苯基-1,2,3,6-四氢吡啶（MPTP）在帕金森病动物模型中产生的缺陷相同，进一步支持了帕金森病可能是由一种作用类似于MPTP的环境毒素引起的这一观点。
