Stuart Jeffrey A, Brown Melanie F
Department of Biological Sciences, Brock University, St. Catharines, Ontario, Canada L2S 3A1.
Biochim Biophys Acta. 2006 Feb;1757(2):79-89. doi: 10.1016/j.bbabio.2006.01.003. Epub 2006 Jan 24.
Oxidative phosphorylation requires assembly of the protein products of both mitochondrial and of nuclear genomes into functional respiratory complexes. Cellular respiration can be compromised when mitochondrial DNA (mtDNA) sequences are corrupted. Oxidative damage resulting from reactive oxygen species (ROS) produced during respiration is probably a major source of mitochondrial genomic instability leading to respiratory dysfunction. Here, we review mechanisms of mitochondrial ROS production, mtDNA damage and its relationship to mitochondrial dysfunction. We focus particular attention on the roles of mtDNA repair enzymes and processes by which the integrity of the mitochondrial genome is maintained and dysfunction prevented.
氧化磷酸化需要将线粒体基因组和核基因组的蛋白质产物组装成功能性呼吸复合体。当线粒体DNA（mtDNA）序列受损时，细胞呼吸可能会受到影响。呼吸过程中产生的活性氧（ROS）导致的氧化损伤可能是线粒体基因组不稳定从而导致呼吸功能障碍的主要来源。在这里，我们综述了线粒体ROS产生、mtDNA损伤的机制及其与线粒体功能障碍的关系。我们特别关注mtDNA修复酶的作用以及维持线粒体基因组完整性和预防功能障碍的过程。
