Li F S
Liaoning Institute of Basic Medical Sciences, Shenyang.
Zhonghua Bing Li Xue Za Zhi. 1990 Jun;19(2):136-8.
Keshan disease (KD) is an endemic cardiomyopathy with membrane damage of the myocardial mitochondria. This paper reports the results of feeding guinea pigs with a forage obtained from KD endemic area. The selenium (Se) content and glutathione peroxidase activity in blood, tissues, mitochondria, microsome and supernatant of myocardium and liver cells of the experimental group were decreased. Additionally, the activity of glutathione-S-transferase was also decreased to some extent, instead of being increased in normal cases, and the content of lipid peroxide and hydroperoxide was increased. At the same time, cardiolipin in mitochondria, and the activity of cytochrome C oxidase (CCO, an enzyme binding with mitochondrial membrane) were also reduced accompanied with oxidative damage and disorder of function of the membrane. Anyhow, when exogenous cardiolipin was added in the purified endemic CCO solution, it was capable of restoring CCO activity significantly. The significance of Se deficiency and unbalanced diet in pathogenesis of Keshan disease is discussed.
克山病(KD)是一种伴有心肌线粒体膜损伤的地方性心肌病。本文报道了用取自克山病病区的一种草料喂养豚鼠的结果。实验组豚鼠血液、组织、心肌和肝细胞线粒体、微粒体及上清液中的硒(Se)含量和谷胱甘肽过氧化物酶活性降低。此外,谷胱甘肽-S-转移酶的活性也有一定程度降低,而非在正常情况下升高,并且脂质过氧化物和氢过氧化物的含量增加。同时,线粒体中的心磷脂以及细胞色素C氧化酶(CCO,一种与线粒体膜结合的酶)的活性也降低,伴有膜的氧化损伤和功能紊乱。总之,当在纯化的病区CCO溶液中添加外源性心磷脂时,它能够显著恢复CCO活性。文中讨论了硒缺乏和饮食不均衡在克山病发病机制中的意义。