Mitochondrial DNA maintenance: an appraisal

Mitochondria play a crucial role in a variety of cellular processes ranging from energy metabolism, generation of reactive oxygen species, and Ca2+ handling to stress responses, cell survival, and death

Alexander T. Akhmedov

2015

Scholarcy highlights

  • Mitochondria play a crucial role in a variety of cellular processes ranging from energy metabolism, generation of reactive oxygen species, and Ca2+ handling to stress responses, cell survival, and death
  • Malfunction of the organelle may contribute to the pathogenesis of neuromuscular disorders, cancer, premature aging, and cardiovascular diseases, including myocardial ischemia, cardiomyopathy, and heart failure
  • Mitochondria are unique as they contain their own genome organized into DNA–protein complexes, so-called mitochondrial nucleoids, along with multiprotein machineries, which promote mitochondrial DNA replication, transcription, and repair
  • The organelle possesses almost all known nuclear DNA repair pathways, including base excision repair, mismatch repair, and recombinational repair, the proximity of mitochondrial DNA to the main sites of ROS production and the lack of protective histones may result in increased susceptibility to oxidative stress and other types of mtDNA damage
  • This review will focus on the mechanisms of mtDNA maintenance with emphasis on the proteins implicated in these processes and their functional role in various disease conditions and aging

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