Akbari Mansour, Nilsen Hilde Loge, Montaldo Nicola Pietro
Department of Medical Biology, Faculty of Health Sciences, UiT-The Arctic University of Norway, Tromsø, Norway.
Department of Clinical Molecular Biology, Institute of Clinical Medicine, University of Oslo, Oslo, Norway.
Front Cell Dev Biol. 2022 Sep 7;10:984245. doi: 10.3389/fcell.2022.984245. eCollection 2022.
Mitochondria are the primary sites for cellular energy production and are required for many essential cellular processes. Mitochondrial DNA (mtDNA) is a 16.6 kb circular DNA molecule that encodes only 13 gene products of the approximately 90 different proteins of the respiratory chain complexes and an estimated 1,200 mitochondrial proteins. MtDNA is, however, crucial for organismal development, normal function, and survival. MtDNA maintenance requires mitochondrially targeted nuclear DNA repair enzymes, a mtDNA replisome that is unique to mitochondria, and systems that control mitochondrial morphology and quality control. Here, we provide an overview of the current literature on mtDNA repair and transcription machineries and discuss how dynamic functional interactions between the components of these systems regulate mtDNA maintenance and transcription. A profound understanding of the molecular mechanisms that control mtDNA maintenance and transcription is important as loss of mtDNA integrity is implicated in normal process of aging, inflammation, and the etiology and pathogenesis of a number of diseases.
线粒体是细胞能量产生的主要场所，许多基本细胞过程都需要线粒体参与。线粒体DNA（mtDNA）是一个16.6 kb的环状DNA分子，仅编码呼吸链复合物约90种不同蛋白质中的13种基因产物以及约1200种线粒体蛋白质。然而，mtDNA对于机体发育、正常功能和生存至关重要。mtDNA的维持需要定位于线粒体的核DNA修复酶、线粒体特有的mtDNA复制体以及控制线粒体形态和质量控制的系统。在此，我们概述了当前关于mtDNA修复和转录机制的文献，并讨论这些系统的组成部分之间动态的功能相互作用如何调节mtDNA的维持和转录。深入了解控制mtDNA维持和转录的分子机制很重要，因为mtDNA完整性的丧失与衰老、炎症的正常过程以及多种疾病的病因和发病机制有关。
