MRC-Mitochondrial Biology Unit, Wellcome Trust/MRC Building, Cambridge, UK.
Nat Rev Mol Cell Biol. 2012 Oct;13(10):659-71. doi: 10.1038/nrm3439. Epub 2012 Sep 20.
Mitochondrial DNA (mtDNA) faces the universal challenges of genome maintenance: the accurate replication, transmission and preservation of its integrity throughout the life of the organism. Although mtDNA was originally thought to lack DNA repair activity, four decades of research on mitochondria have revealed multiple mtDNA repair pathways, including base excision repair, single-strand break repair, mismatch repair and possibly homologous recombination. These mtDNA repair pathways are mediated by enzymes that are similar in activity to those operating in the nucleus, and in all cases identified so far in mammals, they are encoded by nuclear genes.
线粒体 DNA（mtDNA）面临着基因组维护的普遍挑战：在生物体的整个生命周期中，准确复制、传递和保持其完整性。尽管最初认为 mtDNA 缺乏 DNA 修复活性，但对线粒体的四十年研究揭示了多种 mtDNA 修复途径，包括碱基切除修复、单链断裂修复、错配修复和可能的同源重组。这些 mtDNA 修复途径由与核内活性相似的酶介导，并且迄今为止在哺乳动物中鉴定的所有情况中，它们都由核基因编码。
