title: The exonuclease activity of the yeast mitochondrial DNA polymerase γ suppresses mitochondrial DNA deletions between short direct repeats in Saccharomyces cerevisiae.
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.
authors.affiliation: Laboratory of Molecular Genetics, National Institute of Environmental Health Sciences, National Institutes of Health, Research Triangle Park, NC 27709, USA.
authors.forename: Jeffrey D
authors.initials: JD
authors.lastname: Stumpf
authors.forename: William C
authors.initials: WC
authors.lastname: Copeland
mesh_terms: D004256:DNA Polymerase I / Q000235:genetics / Q000378:metabolism*; D004271:DNA, Fungal / Q000032:analysis; D004272:DNA, Mitochondrial / Q000032:analysis*; D018895:Microsatellite Repeats / Q000235:genetics*; D008928:Mitochondria / Q000201:enzymology* / Q000235:genetics; D012441:Saccharomyces cerevisiae / Q000201:enzymology / Q000235:genetics / Q000378:metabolism*; D029701:Saccharomyces cerevisiae Proteins / Q000235:genetics / Q000378:metabolism*; D017384:Sequence Deletion
chemical_list: D004271:DNA, Fungal; D004272:DNA, Mitochondrial; D029701:Saccharomyces cerevisiae Proteins; D004256:DNA Polymerase I; C068352:MIP1 protein, S cerevisiae
keywords: Mip1; Pol Gamma; direct repeats; exonuclease; mtDNA deletions