Mitochondrial respiratory complex I: structure, function and implication in human diseases.

Mitochondrial respiratory complex I: structure, function and implication in human diseases.
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DOI:
10.2174/092986709787846578
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发表时间:
2009
影响因子:
4.1
通讯作者:
Bai Y
Bai Y
中科院分区:
医学3区
文献类型:
--
作者:
Sharma LK;Lu J;Bai Y

文献摘要

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线粒体是真核细胞中普遍存在的细胞器,其主要功能是通过氧化磷酸化以 ATP 形式产生能量供应。作为大多数电子进入呼吸链的入口点,NADH:泛醌氧化还原酶或复合物 I,是线粒体氧化磷酸化系统中最大且最不为人所知的组成部分。近年来,人们在了解其亚基组成、组装、复合物 I 与其他呼吸成分之间的相互作用及其在氧化应激和细胞凋亡中的作用方面取得了实质性进展。这篇综述提供了复合物 I 结构及其细胞功能的最新概述,并讨论了复合物 I 功能障碍在各种人类疾病中的含义。
Mitochondria are ubiquitous organelles in eukaryotic cells whose primary function is to generate energy supplies in the form of ATP through oxidative phosphorylation. As the entry point for most electrons into the respiratory chain, NADH:ubiquinone oxidoreductase, or complex I, is the largest and least understood component of the mitochondrial oxidative phosphorylation system. Substantial progress has been made in recent years in understanding its subunit composition, its assembly, the interaction among complex I and other respiratory components, and its role in oxidative stress and apoptosis. This review provides an updated overview of the structure of complex I, as well as its cellular functions, and discusses the implication of complex I dysfunction in various human diseases.