克山病的分子心脏病学研究轨迹  

The Locus of Molecular Cardiology Research on Keshan Disease

作　　者：杨同书[1] 朱平[1] 颜炜群[1] 周余来[1] 罗贵民[1] 朱筱娟[1] 

机构地区：[1]吉林大学地方病研究所,长春市130021

出　　处：《中国分子心脏病学杂志》2013年第5期695-699,共5页Molecular Cardiology of China

基　　金：国家"七五";"八五"医学科技攻关专题(85-917-01-01)等

摘　　要：目的克山病(Keshan Disease,KD)是在我国16个省区流行的地方性心肌病。几十年来,我国生物化学界对于KD的发病机理做了大量创新性研究.本文回顾和评述我国克山病心脏代谢为中心的研究发展历程。方法和结果包括:使用综合酶谱早期诊断KD的心肌受损;发现KD有低硒性代谢紊乱;发现KD死亡病人心脏有线粒体氧化磷酸化、呼吸酶功能障碍和心肌收缩蛋白结构和功能异常;发现心肌钙代谢紊乱与心肌收缩障碍之间关系;发现KD心肌细胞膜和线粒体膜受损;发现心肌自由基堆积等障碍;发现红细胞的形态、膜脂组分和功能异常;提出KD是一种"心肌线粒体病",或者是一种"膜疾病"等等。通过长期的KD生化研究并与病理、临床所见综合分析,确立了"KD是一种以心肌线粒体损伤为主要特征的原发性代谢性心肌病"的发病机理。提出应用抗氧化药物预防和控制KD策略:应用无机硒、辅酶Q10、含硒模拟物(PZ51)、GPX抗体酶等防治研究KD,取得巨大进展;还证明GPX抗体酶(世界上第一个含硒抗体酶)、PZ51、无机硒、辅酶Q10等对于心肌线粒体自由基损害都有明显的阻止作用。结论克山病是世界上发病最多、研究得最为深入的人体心肌病。这些创新成果为人类的健康做出了贡献,获得国家科技进步奖等奖励。Objective This article reviews and comments on the molecular cardiology research of Keshan disease（KD）. KD is an endemic cardiomyopathy in our country. The reason still unknown, serious threat to people＇s health. Methods and Results For decades, China＇s biochemists through KD research have obtained a lot of innovation achievement. Including： The use of the integrated enzyme spectrum for detect myocardial damage in its early stage and early diagnosis of KD ; Found that KD have tow selenium metabolic disorder; Found that KD died patient＇s heart have that mitochondrial oxidative phosphorylation and respiratory enzyme function obstacle; Myocardial contractite protein structure and function abnormal; Red cell membrane and mitochondrial membrane damage; Puts forward the KD is a kind of＂ mitochondrial cardiomyopathy＂ ,or is a kind of ＂membrane disease＂ and so on.Through the long-term biochemical research of KD, established ＂KD is a kind of primary metabolic cardiomyopathy with mitochondria injuries as its main characteristics＂ pathogenesis. Further proposed that application antionidant drugs as a strategy to prevent and control of KD： Application of inorganic selenium, coenzyme Q10, selenium containing mimics （PZ51）, GPX abzyme etc used to prevent and control the KD and made great advances; Also proved the GPX abzyme （the world＇s first to selenium containing abzyme）, PZS1, inorganic selenium, coenzyme QI0, etc for myocardial mitochondrial free radical damage has obvious protected action. ConcluSion Keshan disease is the world＇s most predominant human cardiomyopmhy. These innovation achievements for human health has made the contribution, won the national science and technology progress prize.

关 键 词：克山病 心脏代谢 线粒体 自由基 

分 类 号：R542.3[医药卫生—心血管疾病]