Scheibye-Knudsen Morten, Fang Evandro F, Croteau Deborah L, Wilson David M, Bohr Vilhelm A
Laboratory of Molecular Gerontology, National Institute on Aging (NIA), National Institutes of Health (NIH), Baltimore, MD 21224, USA.
Laboratory of Molecular Gerontology, National Institute on Aging (NIA), National Institutes of Health (NIH), Baltimore, MD 21224, USA.
Trends Cell Biol. 2015 Mar;25(3):158-70. doi: 10.1016/j.tcb.2014.11.002. Epub 2014 Dec 11.
Mitochondria are the oxygen-consuming power plants of cells. They provide a critical milieu for the synthesis of many essential molecules and allow for highly efficient energy production through oxidative phosphorylation. The use of oxygen is, however, a double-edged sword that on the one hand supplies ATP for cellular survival, and on the other leads to the formation of damaging reactive oxygen species (ROS). Different quality control pathways maintain mitochondria function including mitochondrial DNA (mtDNA) replication and repair, fusion-fission dynamics, free radical scavenging, and mitophagy. Further, failure of these pathways may lead to human disease. We review these pathways and propose a strategy towards a treatment for these often untreatable disorders.
线粒体是细胞中消耗氧气的发电厂。它们为许多重要分子的合成提供了关键环境，并通过氧化磷酸化实现高效的能量产生。然而，氧气的使用是一把双刃剑，一方面为细胞存活提供ATP，另一方面导致有害活性氧（ROS）的形成。不同的质量控制途径维持线粒体功能，包括线粒体DNA（mtDNA）复制和修复、融合-分裂动态、自由基清除和线粒体自噬。此外，这些途径的失效可能导致人类疾病。我们综述这些途径，并提出一种针对这些通常难以治疗的疾病的治疗策略。
