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Mitochondria from myopathies, dystonia, cancer patient samples show frequent mtDNA mutations such as point mutations, insertions and large-scale deletions that could account for mitochondria-associated disease pathogenesis. The mechanism by which such mutations arise following exposure to various DNA-damaging agents is not well understood. One of the well-studied repair pathways in mitochondria is base excision repair. Other repair pathways such as mismatch repair, homologous recombination and microhomology-mediated end joining have also been reported. Interestingly, nucleotide excision repair and classical nonhomologous DNA end joining are not detected in mitochondria. In this review, we summarize the potential causes of mitochondrial genome fragility, their implications as well as various DNA repair pathways that operate in mitochondria.<\/jats:p>","DOI":"10.1042\/bcj20200920","type":"journal-article","created":{"date-parts":[[2021,3,20]],"date-time":"2021-03-20T03:38:56Z","timestamp":1616211536000},"page":"1179-1197","update-policy":"https:\/\/doi.org\/10.1042\/crossmark_policy","source":"Crossref","is-referenced-by-count":22,"title":["Mitochondrial genome stability in human: understanding the role of DNA repair pathways"],"prefix":"10.1042","volume":"478","author":[{"given":"Sumedha","family":"Dahal","sequence":"first","affiliation":[{"name":"Department of Biochemistry, Indian Institute of Science, Bangalore 560012, India"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-3003-1417","authenticated-orcid":false,"given":"Sathees C.","family":"Raghavan","sequence":"additional","affiliation":[{"name":"Department of Biochemistry, Indian Institute of Science, Bangalore 560012, India"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"288","published-online":{"date-parts":[[2021,3,19]]},"reference":[{"key":"2021112218195974300_BCJ-2020-0920C1","doi-asserted-by":"publisher","first-page":"514","DOI":"10.1073\/pnas.94.2.514","article-title":"Mitochondrial DNA damage is more extensive and persists longer than nuclear DNA damage in human cells following oxidative stress","volume":"94","year":"1997","journal-title":"Proc. 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