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Related Concept Videos

Overview of DNA Repair02:25

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In order to be passed through generations, genomic DNA must be undamaged and error-free. However, every day, DNA in a cell undergoes several thousand to a million damaging events by natural causes and external factors. Ionizing radiation such as UV rays, free radicals produced during cellular respiration, and hydrolytic damage from metabolic reactions can alter the structure of DNA. Damages caused include single-base alteration, base dimerization, chain breaks, and cross-linkage.
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Related Experiment Video

Updated: Mar 31, 2026

Using Next Generation Sequencing to Identify Mutations Associated with Repair of a CAS9-induced Double Strand Break Near the CD4 Promoter
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Minimizing the damage: repair pathways keep mitochondrial DNA intact.

Lawrence Kazak1, Aurelio Reyes, Ian J Holt

  • 1MRC-Mitochondrial Biology Unit, Wellcome Trust/MRC Building, Cambridge, UK. lk303@mrc-mbu.cam.ac.uk

Nature Reviews. Molecular Cell Biology
|September 21, 2012
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Mitochondrial DNA (mtDNA) maintenance involves complex repair pathways, including base excision repair and mismatch repair, despite initial beliefs of its lack of DNA repair activity. These essential repair mechanisms are encoded by nuclear genes in mammals.

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Area of Science:

  • Mitochondrial biology
  • Molecular genetics
  • DNA repair

Background:

  • Mitochondrial DNA (mtDNA) requires robust maintenance for organismal health.
  • Historically, mtDNA was believed to lack intrinsic DNA repair capabilities.
  • Decades of research have uncovered sophisticated mtDNA maintenance mechanisms.

Purpose of the Study:

  • To elucidate the existence and nature of DNA repair pathways within mitochondria.
  • To identify the enzymes and genetic origins of mtDNA repair systems.

Main Methods:

  • Review of existing literature on mitochondrial DNA repair.
  • Analysis of enzymatic activities in mitochondria.
  • Investigation of gene origins for mtDNA repair enzymes.

Main Results:

  • Multiple mtDNA repair pathways have been identified, including base excision repair, single-strand break repair, and mismatch repair.
  • Evidence suggests homologous recombination may also occur in mtDNA.
  • Mitochondrial repair enzymes exhibit functions analogous to nuclear repair enzymes.
  • All identified mammalian mtDNA repair enzymes are encoded by nuclear genes.

Conclusions:

  • Mitochondria possess active and diverse DNA repair pathways crucial for genome integrity.
  • Nuclear genes play a vital role in encoding the enzymatic machinery for mitochondrial DNA repair.