人线粒体DNA复制与转录调控

Replication-transcription regulation of human mitochondrial DNA

线粒体是真核细胞能量代谢、物质代谢和信号调控的核心细胞器。线粒体DNA(mitochondrial DNA,mtDNA)的复制与转录调控对维持正常的线粒体功能至关重要。研究表明,线粒体转录延伸因子(mitochondrial transcription elongation factor,TEFM)和线粒体DNA单链结合蛋白(mitochondrial DNA single-strand binding protein,mtSSB)作为调控mtDNA复制与转录之间转换的分子开关,实现特异性调控mtDNA的维持和表达。本文就TEFM和mtSSB对mtDNA复制与转录的调控作简要总结,以期为相关疾病的致病机制和干预研究提供思路。

Mitochondria are the key organelles in energy production, cell metabolism and signaling. The precise regulation of mitochondrial DNA(mtDNA) replication and transcription plays a critical role in the maintenance of mitochondrial functions. It has been shown that mitochondrial transcription elongation factor(TEFM) and mitochondrial DNA single-strand binding protein(mtSSB) act as molecular switches between mtDNA replication and transcription, allowing specific regulation of mtDNA maintenance and expression. In this review, we briefly summarize the recent advances in replication-transcription regulation of human mitochondrial DNA by TEFM and mtSSB in order to provide insights into the molecular mechanism and intervention strategy of diseases caused by the deficiency of TEFM and mtSSB.