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Genetics. 2013 Jun;194(2):519-22. doi: 10.1534/genetics.113.150920. Epub 2013 Apr 15.

The exonuclease activity of the yeast mitochondrial DNA polymerase γ suppresses mitochondrial DNA deletions between short direct repeats in Saccharomyces cerevisiae.

Author information

1
Laboratory of Molecular Genetics, National Institute of Environmental Health Sciences, National Institutes of Health, Research Triangle Park, NC 27709, USA.

Abstract

The importance of mitochondrial DNA (mtDNA) deletions in the progeroid phenotype of exonuclease-deficient DNA polymerase γ mice has been intensely debated. We show that disruption of Mip1 exonuclease activity increases mtDNA deletions 160-fold, whereas disease-associated polymerase variants were mostly unaffected, suggesting that exonuclease activity is vital to avoid deletions during mtDNA replication.

KEYWORDS:

Mip1; Pol Gamma; direct repeats; exonuclease; mtDNA deletions

PMID:
23589460
PMCID:
PMC3664861
DOI:
10.1534/genetics.113.150920
[Indexed for MEDLINE]
Free PMC Article
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