线粒体电子传递链复合物Ⅲ和线粒体疾病  

Complex Ⅲ of the electron transport chain and mitochondrial diseases

作　　者：崔铁忠[1] 卢康荣[2] 李银霞[3] 王万山[4] 

机构地区：[1]耶鲁大学医学院细胞生物学系,美国纽黑文06520 [2]南方医科大学基础医学院,广东广州510515 [3]犹他大学医学院肿瘤科学系,美国盐湖城84112 [4]南方医科大学比较医学研究所,广东广州510515

出　　处：《科技视界》2016年第11期38-40,共3页Science & Technology Vision

基　　金：广东省科技计划项目(No.2011B060300028;2012B040304010)

摘　　要：线粒体是细胞内不可或缺的细胞器,它合成细胞所需90%以上的能量。线粒体疾病多为遗传性罕见病,但其致死性强且致病机理还不清晰。电子传递链复合物Ⅲ是电子传递的重要载体,与呼吸链上其他复合物相比,其与线粒体疾病的联系并不是很清楚。随着人们对复合物Ⅲ组装过程研究的不断深入,多个致病基因不断被发现,使得人们重新审视其与线粒体疾病的关系。本综述以近年来复合物Ⅲ的研究进展为基础,分析并总结了相关线粒体疾病的发病机理和未来的研究方向。Mitochondrion is an essential organelle which produces above 90% of energy in cells. Mitochondrial diseases are rare genetic disorders,but they are potentially life-threatening diseases with unknown pathogenic mechanisms. Complex Ⅲ（also called Cytochrome c reductase） is directly involved in the electron transport in the mitochondria. Relative to the other respiratory complexes, the complex Ⅲ is loosely associated with mitochondrial diseases. With the rapid expanding of complex Ⅲ knowledge in the recent years, multiple mutations in the genes of complex Ⅲ have been discovered to cause mitochondrial diseases, and this also calls attentions of scientists on the relation between complex Ⅲ and mitochondrial diseases. Based upon the recent progress of mitochondrial complex Ⅲ, we summarized the complex Ⅲ-associated mitochondrial diseases and the future research directions.

关 键 词：线粒体 疾病 复合物Ⅲ 

分 类 号：Q244[生物学—细胞生物学]