Mitochondrial DNA replication and repair: all a flap

Trends Biochem Sci. 2009 Jul;34(7):358-65. doi: 10.1016/j.tibs.2009.03.007. Epub 2009 Jun 24.


The mitochondrial genome is dwarfed by its neighbour in the nucleus, and, thus, it has been sensible for far more resources to be invested in the study of nuclear, rather than mitochondrial, DNA metabolism. Furthermore, few researchers have considered using mitochondrial DNA (mtDNA) as a model system for nuclear DNA metabolism. A quick look into the history of mtDNA provides ready answers as to why this was the case; however, recently mitochondria have been found to contain several nuclear replication and repair factors, so is there any potential to adopt the mitochondrion as a tool to unravel some of the intricacies of replication and repair in higher-order eukaryotes? Perhaps it is now time to invite the Cinderella genome to the ball.

Publication types

  • Research Support, Non-U.S. Gov't
  • Review

MeSH terms

  • Animals
  • DNA / metabolism
  • DNA Repair Enzymes / genetics
  • DNA Repair Enzymes / metabolism
  • DNA Repair*
  • DNA Replication*
  • DNA, Mitochondrial / biosynthesis
  • DNA, Mitochondrial / metabolism*
  • Humans
  • Mitochondria / metabolism*
  • Models, Biological


  • DNA, Mitochondrial
  • Okazaki fragments
  • DNA
  • DNA Repair Enzymes