MITOCHONDRIAL COMPLEX I DEFICIENCY IN PARKINSONS-DISEASE
MITOCHONDRIAL COMPLEX I DEFICIENCY IN PARKINSONS-DISEASE
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DOI：
10.1111/j.1471-4159.1990.tb02325.x
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发表时间：
1990-03-01
影响因子：
4.7
通讯作者：
MARSDEN, CD
中科院分区：
文献类型：
--
作者：
SCHAPIRA, AHV;COOPER, JM;MARSDEN, CD
研究了9例帕金森病患者和9例正常对照者死后黑质线粒体代谢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（复合物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物I活性的特定缺陷。这种生物化学缺陷与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的环境毒素引起的主张。
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.