中文摘要：

从线粒体DNA复制的模型与机制、复制的调控、复制忠实性及其损伤修复3个方面对近年来的研究文献进行了总结.在复制的模型与机制方面,对传统的D环复制的细节有了更深入的了解,新的实验方法的结果显示,在哺乳动物中还存在着链结合单向复制和链结合双向复制2种模型.在线粒体DNA复制的调控方面,近年来研究较多的调控因子主要包括mtDNA聚合酶γ、线粒体单链结合蛋白（mtSSB）、引物酶、解旋酶、连接酶、拓扑异构酶、转录因子mtTFA等,介绍了这些因子的最新研究进展及调控机制;对mtDNA复制时期和拷贝数量调控机制的研究也有突破,确定了Abf2p是mtDNA复制时期与拷贝数目的调控因子.在mtDNA复制的忠实性及其损伤修复研究方面,主要涉及到DNA Polγ的校正功能、错配修复、重组修复、DNA切除修复等,在mtDNA损伤修复中仅存在碱基切除修复机制,缺少核苷酸切除修复机制.

英文摘要：

The recent research progress of mitochondrial DNA replication models and mechanisms, its regulation, fidelity of mtDNA replication and its damage repair systems were reviewed. With a respect of models and mechanisms of mitochondrial DNA replication, traditional strand-displacement model has been made a great progress in details. The results from a new experimental method shown that there were two other models, strand-coupled unidirectional replication and strand-coupled bidirectional replication, exist in mammal mitochondria. With a respect of mitochondrial DNA replication rcgulation, most researches focused on mtDNA Polγ, mitochondrial single-stranded DNA binding protein （mtSSB）, primerase, helicase, ligase, topoisomerase, and transcription factor mtTFA. The progress and action mechanisms of these regulatory factors were surveyed in details. The researches on period of mtDNA replication and the regulation mechanisms of mtDNA copy number also have been made a great progress, and a main regulatory factor Abf2p was identified. Finally, the fidelity of mtDNA replication and its damage repair system concern with correction function of DNA Polγ, mismatch repair, recombination repair, and DNA excision repair. In mitochondria only base excision repair （BER） has proven to exist, nucleotide excision repair （NER） has not proven so far.