摘要:大量证据表明,半自治细胞器线粒体在许多神经退行性疾病的进展中起关键作用。线粒体DNA(mtDNA)编码Oxphos复合物的成分,但突变的mtDNA用AGING积聚在细胞中,这反映了神经退行性疾病的患病率的增加。这种积累不仅源于mtDNA和高度氧化环境的修复,而且还源于裂变后的有效线粒体。In this review, we focus on several pivotal mitochondrial proteins related to mtDNA maintenance (such as ATAD3A and TFAM), mtDNA alterations including mtDNA mutations, mtDNA elimination, and mtDNA release-activated inflammation to understand the crucial role played by mtDNA in the pathogenesis of neurodegenerative diseases such as Alz- heimer's disease,帕金森氏病,肌萎缩性侧索硬化和亨廷顿氏病。我们的工作概述了针对mtDNA的新型治疗策略。