Oxidative Damage and Repair in the Mitochondrial Genome

  • Lene Juel Rasmussen
  • Keshav K. Singh
Part of the Molecular Biology Intelligence Unit book series (MBIU)


DNA is constantly exposed to damaging agents from both endogenous and exogenous sources. If this damage is not repaired, it can lead to mutations and result in cellular dysfunction, including uncontrolled cell proliferation. Thus, in order to maintain the integrity of the genome, a complex network of DNA repair pathways exists to remove the majority of deleterious lesions. However, DNA repair may occasionally fail or become limited due to an excess DNA damage, resulting in DNA damage accumulation.

Characterization of DNA repair mechanisms has generally focused on these processes in nuclear DNA. While the interest and research efforts concerning DNA repair in mitochondria have increased, much is still to be learned. In this chapter, we will focus on current knowledge of oxidative DNA damage and repair systems in the mitochondrion, make comparisons to what is known about repair in nucleus, as well as discuss the link between dysfunctional mitochondria and human disease.


Mismatch Repair Base Excision Repair Mitochondrial Target Sequence Translesion Synthesis Thymine Glycol 


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Copyright information

© Landes Bioscience and Springer Science+Business Media 2007

Authors and Affiliations

  • Lene Juel Rasmussen
    • 2
  • Keshav K. Singh
    • 1
  1. 1.Department of Cancer GeneticsRoswell Park Cancer InstituteBuffaloUSA
  2. 2.Department of Life Sciences and ChemistryRoskilde UniversityRoskildeDenmark

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