核心期刊网首页> 中文期刊> 基础科学

Keshan disease and mitochondrial cardiomyopathy


Keshan disease (KD) is a potentially fatal form of cardiomyopathy (disease of the heart muscle) endemic in certain areas of China. From 1984 to 1986, a national comprehensive scientific investigation on KD in Chuxiong region of Yunnan Province in the southwest China was conducted. The investigation team was composed of epidemiologists, clinic doctors, pathologists, biochemists, biophysicists and specialists in ecological environment. Results of pathological, biochemical and biophysical as well as clinical studies showed: an obvious increase of enlarged and swollen mitochondria with distended crista membranes in myocardium from patients with KD; significant reductions in the activity of oxidative phosphorylation (succinate dehydrogenase, cytochrome oxidase, succinate oxidase, H+-ATPase) of affected mitochondria; decrease in CoQ, cardiolipin, Se and GSHPx activity, while obvious increase in the Ca2+ content. So, it was suggested that mitochondria are the predominant target of the pathogenic factors of KD. Before Chuxiong KD survey only a few cases of mitochondrial cardiomyopathy were studied. During the multidisciplinary scientific investigation on KD in Chuxiong a large amount of samples from KD cases and the positive controls were examined. On the basis of the results obtained it was suggested that KD might be classified as a 'Mitochondrial Cardiomyopathy' endemic in China. This is one of the achievements in the three years' survey in Chuxiong and is valuable not only to the deeper understanding of pathogenic mechanism of KD but also to the study of mitochondrial cardiomyopathy in general.Keshan disease is not a genetic disease, but is closely related to the malnutrition (especially microelement Se deficiency). KD occurs along a low Se belt, and Se supplementation has been effective in prevention of such disease. The incidence of KD has sharply decreased along with the steady raise of living standard and realization of preventive measures. At present, patients of KD are very sparse. In recent years the research on the non-KD mitochondrial cardiomyopathy has progressed rapidly. Given the advances in this aspect a minireview is written to evaluate the classification of KD as a kind of mitochondrial cardiomyopathy.......

【作者名称】: YANG Fuyu
【作者单位】: National Laboratory of Biomacromolecules, Institute of Biophysics, Chinese Academy of Sciences, Beijing 100101, China
【关 键 词】: mitochondrial disease, Keshan disease, mitochondrial cardiomyopathy
【期刊名称】: 中国科学:生命科学(英文版)
【期刊论文数据库】: [DBS_Articles_01]
【期刊论文编号】: 105,648,723
【摘要长度】: 2,251
【上篇论文】: 中文期刊 - Improving Indigenous Technologies for Sustainable Land Use in Northern Mountainous Areas of Vietnam
【下篇论文】: 中文期刊 - Geographical characteristics of China's wetlands derived from remotely sensed data

【论文下载】: 免费获取 该期刊&论文全文内容