Piccolo G, Banfi P, Azan G, Rizzuto R, Bisson R, Sandoná D, Bellomo G
Fondazione Istituto Neurologico C. Mondino, Clinica Neurologica II, Pavia, Italy.
J Neurol Sci. 1991 Sep;105(1):57-60. doi: 10.1016/0022-510x(91)90118-q.
According to experimental models suggesting that overproduction of oxygen free-radicals may occur when the electron transport in the respiratory chain is impaired, we searched for in vivo biological markers of oxidative stress in 11 patients affected by histologically proven mitochondrial myopathy with progressive external ophthalmoplegia (PEO) and partial cytochrome c oxidase deficiency in muscle fibres. Six of the patients carried large-scale deletions of mitochondrial DNA. Biochemical assays included the determination of plasma and erythrocyte reduced glutathione (GSH) concentration, plasma malondialdehyde, fluorescent adducts of aldehydes with plasma proteins, and serum level of lipid peroxides. In patients with PEO the mean values of lipid peroxides and of the fluorescent adducts of aldehydes with plasma proteins were significantly higher with respect to normal controls, while the mean values of plasma and erythrocyte GSH concentration were significantly lower. The reported data indicate an increase of lipid peroxidation indexes along with the reduction of one of the most important antioxidant systems and suggest the hypothesis that overproduction of reduced oxygen species might be an adjunctive cause of cell damage in mitochondrial myopathies and encephalomyopathies associated with defects of oxidative phosphorylation.
根据实验模型表明，当呼吸链中的电子传递受损时，可能会发生氧自由基的过度产生，我们在11例经组织学证实患有线粒体肌病伴进行性眼外肌麻痹（PEO）且肌纤维部分细胞色素c氧化酶缺乏的患者中寻找氧化应激的体内生物学标志物。其中6例患者存在线粒体DNA的大规模缺失。生化检测包括测定血浆和红细胞中还原型谷胱甘肽（GSH）浓度、血浆丙二醛、醛与血浆蛋白的荧光加合物以及脂质过氧化物的血清水平。在患有PEO的患者中，脂质过氧化物以及醛与血浆蛋白的荧光加合物的平均值相对于正常对照组显著更高，而血浆和红细胞GSH浓度的平均值则显著更低。所报道的数据表明脂质过氧化指标增加，同时最重要的抗氧化系统之一减少，并提出了这样的假设：还原型氧物种的过度产生可能是与氧化磷酸化缺陷相关的线粒体肌病和脑肌病中细胞损伤的辅助原因。
