Reichmann H, Janetzky B, Bischof F, Seibel P, Schöls L, Kuhn W, Przuntek H
Department of Neurology, University of Würzburg, Germany.
Eur Neurol. 1994;34(5):263-7. doi: 10.1159/000117053.
There is good evidence that patients with Parkinson's disease have respiratory chain dysfunction in their substantia nigra. Since mitochondrial cytopathies due to enzyme defects in the respiratory chain are predominantly manifested in tissues with a high oxidative metabolism we analyzed oxidative energy metabolism in skeletal muscle from 6 patients with Parkinson's disease. Control muscles were from subjects of the same age group. Histological and histochemical analyses showed no morphological abnormalities found in mitochondrial myopathies. Biochemical analyses of the various complexes of the respiratory chain were normal. Since 13 subunits of complexes I, III, IV and V of the respiratory chain are encoded by the mitochondrial genome we performed Southern blot and PCR analyses in skeletal muscle from patients and controls and found no disease-specific increase in deletions or insertions of the mitochondrial genome. Therefore, we do not think that skeletal muscle reflects the mitochondrial disturbance in Parkinson's disease found in the substantia nigra.
有充分证据表明，帕金森病患者黑质存在呼吸链功能障碍。由于呼吸链中酶缺陷导致的线粒体细胞病变主要表现在高氧化代谢的组织中，我们分析了6例帕金森病患者骨骼肌的氧化能量代谢。对照肌肉取自同一年龄组的受试者。组织学和组织化学分析未发现线粒体肌病中的形态学异常。呼吸链各种复合物的生化分析正常。由于呼吸链复合物I、III、IV和V的13个亚基由线粒体基因组编码，我们对患者和对照者的骨骼肌进行了Southern印迹和PCR分析，未发现线粒体基因组缺失或插入有疾病特异性增加。因此，我们认为骨骼肌不能反映帕金森病患者黑质中发现的线粒体紊乱。
