线粒体呼吸链复合体I-中国细胞生物学学报.pdf

网络出版时间：2014-07-25 09:50 网络出版地址：/kcms/doi/10.11844/cjcb.2014.08.0037.html 中国细胞生物学学报 Chinese Journal of Cell Biology 2014, 36(8): DOI: 10.11844/cj cb.2014.08.0037 线粒体呼吸链复合体I 1 1 1* 1,2* 李凤杰 沈丽君 方合志 白益东 1 2 ( 温州医科大学检验医学院、生命科学学院, 温州325035; 德州大学医学中心, 圣安东尼奥78229, 美国) 摘要 线粒体呼吸链复合体I是呼吸链电子传递的起始复合体, 作为电子传递过程的限速酶, 复 合体I的分子量远大于其余的四个呼吸链复合体。复合体I相关的疾病发生除了与40余个复合体I组成 亚基的突变相关外, 还同参与其组装的多个组装因子存在密切联系。该文对复合体I的结构以及参与 调控复合体I 组装的各类组装因子进行综述, 旨在为全面了解复合体I相关疾病的发生提供具体参考。 关键词 线粒体; 呼吸链复合体I; 组装因子 Mitochondrial Respiratory Complex I 1 1 1* 1,2* Li Fengjie , Shen Lijun , Fang Hezhi , Bai Yidong 1 ( School of Laboratory Medicine and Life Science, Wenzhou Medical University, Wenzhou 325035, China; 2 University of Texas Health Science Center, San Antonio 78229, USA) Abstract Mitochondrial respiratory complex I (NADH: ubiquinone oxidoreductase) is the initial and rate limiting enzyme in electron transfer chain (ETC). In all five respiratory complexes, complex I is the largest enzyme with a molecular weight about 1 MDa. Mutations in both 45 subunits of complex I and its assemble factors have been implicated in many diseases. To provide a comprehensive understanding of complex I in human diseases, the complex I structure and the assembly of complex I were discussed