摘要： 线粒体DNA（mtDNA）编码线粒体电子传递系统的亚单位以及构建翻译机器所需的各种rRAN和tRNA。mtDNA编码的每一个亚单位都是线粒体完成正常的氧化磷酸化过程所必需的，因此，线粒体DNA的完整性对于生物体的生存十分重要。长期以来，人们一直认为线粒体中不存在DNA的修复。近年来在线粒体提取物中却检测到了一定数量的修复因子，提示线粒体中存在DNA的修复。主要对线粒体修复系统中相关酶的研究进展进行综述。
Abstract: Mitochondrial DNA（mtDNA） encodes subunits of the mitochondrial electron transport system and the rRNAs and tRNAs required for constructing the mitochondrial tranlational machinery.Each subunit encoded by mtDNA is essential for normal oxidative phosphorylation.Thus,integrity of the mtDNA is crucial for the survival of organisms.It has long been held that there is no DNA repair in mitochondria.But in recent years,a number of repair factors have been found in mitochondrial extracts,suggesting the presence of DNA repair in mitochondria.This review summarized recent progress of enzyme in mitochondrial DNA repair processes.