DNA ligase III is critical for mtDNA integrity but not Xrcc1-mediated nuclear DNA repair

Yankun Gao, Sachin Katyal, Youngsoo Lee, Jingfeng Zhao, Jerold E. Rehg, Helen R. Russell and Peter J. McKinnon ()
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Yankun Gao: St Jude Children’s Research Hospital
Sachin Katyal: St Jude Children’s Research Hospital
Youngsoo Lee: St Jude Children’s Research Hospital
Jingfeng Zhao: St Jude Children’s Research Hospital
Jerold E. Rehg: St Jude Children’s Research Hospital
Helen R. Russell: St Jude Children’s Research Hospital
Peter J. McKinnon: St Jude Children’s Research Hospital

Nature, 2011, vol. 471, issue 7337, 240-244

Abstract: A mitochondrial role for DNA ligase III Mammalian cells contain three different DNA ligase enzymes, each with different properties but all involved in DNA replication and repair. Ligase III (Lig3) is known to form a complex with the nuclear DNA repair protein Xrcc1, and Lig3 null animals cannot be made. This raises the question of whether this nuclear role in base-excision repair (BER) is the critical function of Lig3 that maintains viability. Two groups reporting in this issue of Nature investigate different aspects of Lig3 function in vivo, both concluding that the catalytic activity of Lig3 is critical for mitochondrial DNA maintenance and viability, but unexpectedly, is dispensable for Xrcc1-mediated nuclear BER. These findings suggest that Lig3 mutations might cause some of the human syndromes associated with defects in the replication and/or repair of mitochondrial DNA.

Date: 2011
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DOI: 10.1038/nature09773

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