Zhongshan Hospital Institute of Clinical Science, Fudan University, Shanghai Medical College, Shanghai, China.
MRC Mitochondrial Biology Unit, University of Cambridge, Cambridge, UK.
Adv Exp Med Biol. 2017;1038:85-105. doi: 10.1007/978-981-10-6674-0_7.
Mitochondria are vital double-membrane organelles that act as a "powerhouse" inside the cell and have essential roles to maintain cellular functions, e.g., ATP production, iron-sulfur synthesis metabolism, and steroid synthesis. An important difference with other organelles is that they contain their own mitochondrial DNA (mtDNA). Such powerful organelles are also sensitive to both endogenous and exogenous factors that can cause lesions to their structural components and their mtDNA, resulting in gene mutations and eventually leading to diseases. In this review, we will mainly focus on mammalian mitochondrial DNA repair pathways that safeguard mitochondrial DNA integrity and several important factors involved in the repair process, especially on an essential pathway, base excision repair. We eagerly anticipate to explore more methods to treat related diseases by constantly groping for these complexes and precise repair mechanisms.
线粒体是重要的双层膜细胞器，作为细胞内的“动力工厂”，在维持细胞功能方面发挥着重要作用，例如，ATP 生成、铁硫合成代谢和类固醇合成。与其他细胞器的一个重要区别是，它们含有自己的线粒体 DNA（mtDNA）。如此强大的细胞器也容易受到内源性和外源性因素的影响，这些因素会导致它们的结构成分和 mtDNA 发生损伤，从而导致基因突变，最终导致疾病。在这篇综述中，我们将主要关注哺乳动物线粒体 DNA 修复途径，这些途径可以保护线粒体 DNA 的完整性，以及修复过程中涉及的几个重要因素，特别是基本的碱基切除修复途径。我们热切期待通过不断探索这些复合物和精确的修复机制，找到更多治疗相关疾病的方法。
