{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,5]],"date-time":"2026-10-05T15:52:37Z","timestamp":1791215557426,"version":"4.1.0"},"reference-count":117,"publisher":"Frontiers Media SA","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Front. Genet."],"DOI":"10.3389\/fgene.2015.00106","type":"journal-article","created":{"date-parts":[[2015,3,17]],"date-time":"2015-03-17T16:39:35Z","timestamp":1426610375000},"source":"Crossref","is-referenced-by-count":27,"title":["DNA polymerase \u00ce\u00b3 and disease: what we have learned from yeast"],"prefix":"10.3389","volume":"6","author":[{"given":"Tiziana","family":"Lodi","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Cristina","family":"Dallabona","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Cecilia","family":"Nolli","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Paola","family":"Goffrini","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Claudia","family":"Donnini","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Enrico","family":"Baruffini","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1965","published-online":{"date-parts":[[2015,3,17]]},"reference":[{"key":"B1","doi-asserted-by":"publisher","first-page":"5585","DOI":"10.1038\/ncomms6585","article-title":"Mitochondrial protein sorting as a therapeutic target for ATP synthase disorders","volume":"5","author":"Aiyar","year":"2014","journal-title":"Nat. Commun"},{"key":"B2","doi-asserted-by":"publisher","first-page":"1212","DOI":"10.1093\/hmg\/ddq565","article-title":"Sequence-specific stalling of DNA polymerase \u00ce\u00b3 and the effects of mutations causing progressive ophthalmoplegia","volume":"20","author":"Atanassova","year":"2011","journal-title":"Hum. Mol. Genet"},{"key":"B3","doi-asserted-by":"publisher","first-page":"2610","DOI":"10.1128\/AAC.01659-08","article-title":"R964C mutation of DNA polymerase gamma imparts increased stavudine toxicity by decreasing nucleoside analog discrimination and impairing polymerase activity","volume":"53","author":"Bailey","year":"2009","journal-title":"Antimicrob. Agents Chemother"},{"key":"B4","doi-asserted-by":"crossref","first-page":"2115","DOI":"10.1002\/j.1460-2075.1984.tb02099.x","article-title":"The initiation of DNA replication in the mitochondrial genome of yeast","volume":"3","author":"Baldacci","year":"1984","journal-title":"EMBO J"},{"key":"B5","doi-asserted-by":"publisher","first-page":"52","DOI":"10.1016\/j.mito.2014.11.003","article-title":"Polymorphisms in DNA polymerase \u00ce\u00b3 affect the mtDNA stability and the NRTI-induced mitochondrial toxicity in Saccharomyces cerevisiae","volume":"20","author":"Baruffini","year":"2015","journal-title":"Mitochondrion"},{"key":"B6","doi-asserted-by":"publisher","first-page":"1225","DOI":"10.1016\/j.bbadis.2007.10.002","article-title":"Mitochondrial DNA defects in Saccharomyces cerevisiae caused by functional interactions between DNA polymerase gamma mutations associated with disease in human","volume":"1772","author":"Baruffini","year":"2007b","journal-title":"Biochim. Biophys. Acta"},{"key":"B7","doi-asserted-by":"publisher","first-page":"426","DOI":"10.1016\/j.ymeth.2010.02.023","article-title":"In vivo analysis of mtDNA replication defects in yeast","volume":"51","author":"Baruffini","year":"2010","journal-title":"Methods"},{"key":"B8","doi-asserted-by":"publisher","first-page":"182","DOI":"10.1016\/j.mito.2010.09.007","article-title":"Predicting the contribution of novel POLG mutations to human disease through analysis in yeast model","volume":"11","author":"Baruffini","year":"2011","journal-title":"Mitochondrion"},{"key":"B9","doi-asserted-by":"publisher","first-page":"183","DOI":"10.1016\/j.mito.2009.10.002","article-title":"Construction and validation of a yeast model system for studying in vivo the susceptibility to nucleoside analogues of DNA polymerase gamma allelic variants","volume":"10","author":"Baruffini","year":"2010","journal-title":"Mitochondrion"},{"key":"B10","doi-asserted-by":"publisher","first-page":"1227","DOI":"10.1534\/genetics.107.079293","article-title":"A single nucleotide polymorphism in the DNA polymerase gamma gene of Saccharomyces cerevisiae laboratory strains is responsible for increased mitochondrial DNA mutability","volume":"177","author":"Baruffini","year":"2007a","journal-title":"Genetics"},{"key":"B11","doi-asserted-by":"publisher","first-page":"2846","DOI":"10.1093\/hmg\/ddl219","article-title":"Genetic and chemical rescue of the Saccharomyces cerevisiae phenotype induced by mitochondrial DNA polymerase mutations associated with progressive external ophthalmoplegia in humans","volume":"15","author":"Baruffini","year":"2006","journal-title":"Hum. Mol. Genet"},{"key":"B12","doi-asserted-by":"publisher","first-page":"e34322","DOI":"10.1371\/journal.pone.0034322","article-title":"Overexpression of DNA polymerase zeta reduces the mitochondrial mutability caused by pathological mutations in DNA polymerase gamma in yeast","volume":"7","author":"Baruffini","year":"2012","journal-title":"PLoS ONE"},{"key":"B13","doi-asserted-by":"publisher","first-page":"219","DOI":"10.1016\/0092-8674(89)90883-0","article-title":"A conserved 3\u00e2\u20ac\u00b2-5\u00e2\u20ac\u00b2 exonuclease active site in prokaryotic and eukaryotic DNA polymerases","volume":"59","author":"Bernad","year":"1989","journal-title":"Cell"},{"key":"B15","doi-asserted-by":"publisher","first-page":"1590","DOI":"10.1093\/hmg\/ddp074","article-title":"In vitro supplementation with dAMP\/dGMP leads to partial restoration of mtDNA levels in mitochondrial depletion syndromes","volume":"18","author":"Bulst","year":"2009","journal-title":"Hum. Mol. Genet"},{"key":"B16","doi-asserted-by":"publisher","first-page":"95","DOI":"10.1016\/j.ymgme.2012.04.022","article-title":"In vitro supplementation with deoxynucleoside monophosphates rescues mitochondrial DNA depletion","volume":"107","author":"Bulst","year":"2012","journal-title":"Mol. Genet. Metab"},{"key":"B17","doi-asserted-by":"publisher","first-page":"36679","DOI":"10.1074\/jbc.274.51.36679","article-title":"Yeast Sml1, a protein inhibitor of ribonucleotide reductase","volume":"17","author":"Chabes","year":"1999","journal-title":"J. Biol. Chem"},{"key":"B18","doi-asserted-by":"publisher","first-page":"312","DOI":"10.1016\/j.bbabio.2008.10.007","article-title":"DNA polymerase gamma and mitochondrial disease: understanding the consequence of POLG mutations","volume":"1787","author":"Chan","year":"2009","journal-title":"Biochim. Biophys. Acta"},{"key":"B19","doi-asserted-by":"publisher","first-page":"1381","DOI":"10.1016\/j.dnarep.2005.08.010","article-title":"Mono-allelic POLG expression resulting from nonsense-mediated decay and alternative splicing in a patient with Alpers syndrome","volume":"4","author":"Chan","year":"2005b","journal-title":"DNA Repair"},{"key":"B20","doi-asserted-by":"publisher","first-page":"31341","DOI":"10.1074\/jbc.M506762200","article-title":"The common A467T mutation in the human mitochondrial DNA polymerase (POLG) compromises catalytic efficiency and interaction with the accessory subunit","volume":"280","author":"Chan","year":"2005a","journal-title":"J. Biol. Chem"},{"key":"B21","doi-asserted-by":"publisher","first-page":"270","DOI":"10.1016\/j.bbrc.2012.12.119","article-title":"Role of polymerase \u00ce\u00b7 in mitochondrial mutagenesis of Saccharomyces cerevisiae","volume":"431","author":"Chatterjee","year":"2013","journal-title":"Biochem. Biophys. Res. Commun"},{"key":"B22","doi-asserted-by":"publisher","first-page":"815","DOI":"10.1038\/nrg1708","article-title":"The organization and inheritance of the mitochondrial genome","volume":"6","author":"Chen","year":"2005","journal-title":"Nat. Rev. Genet"},{"key":"B23","doi-asserted-by":"publisher","first-page":"714","DOI":"10.1126\/science.1106391","article-title":"Aconitase couples metabolic regulation to mitochondrial DNA maintenance","volume":"307","author":"Chen","year":"2005","journal-title":"Science"},{"key":"B24","doi-asserted-by":"publisher","first-page":"742","DOI":"10.1016\/j.ejcb.2010.06.008","article-title":"Association of the yeast DNA helicase Pif1p with mitochondrial membranes and mitochondrial DNA","volume":"89","author":"Cheng","year":"2010","journal-title":"Eur. J. Cell. Biol"},{"key":"B25","doi-asserted-by":"crossref","first-page":"29984","DOI":"10.1016\/S0021-9258(18)43978-6","article-title":"Purification and characterization of MSH1, a yeast mitochondrial protein that binds to DNA mismatches","volume":"269","author":"Chi","year":"1994","journal-title":"J. Biol. Chem"},{"key":"B26","doi-asserted-by":"publisher","first-page":"244","DOI":"10.2174\/157016209787581409","article-title":"Relationship between polymerase gamma (POLG) polymorphisms and antiretroviral therapy-induced lipodystrophy in HIV-1 infected patients: a case-control study","volume":"7","author":"Chiappini","year":"2009","journal-title":"Curr. HIV Res"},{"key":"B27","doi-asserted-by":"publisher","first-page":"457","DOI":"10.1016\/j.molcel.2008.11.007","article-title":"DNA2 resolves expanding flap in mitochondrial base excision repair","volume":"32","author":"Copeland","year":"2008","journal-title":"Mol. Cell"},{"key":"B28","doi-asserted-by":"publisher","first-page":"11989","DOI":"10.1073\/pnas.1101478108","article-title":"A yeast-based assay identifies drugs active against human mitochondrial disorders","volume":"108","author":"Couplan","year":"2011","journal-title":"Proc. Natl. Acad. Sci. U.S.A"},{"key":"B29","doi-asserted-by":"publisher","first-page":"365","DOI":"10.1534\/genetics.109.104497","article-title":"Polymorphisms in multiple genes contribute to the spontaneous mitochondrial genome instability of Saccharomyces cerevisiae S288C strains","volume":"183","author":"Dimitrov","year":"2009","journal-title":"Genetics"},{"key":"B30","doi-asserted-by":"crossref","first-page":"8201","DOI":"10.1128\/MCB.19.12.8201","article-title":"Dual role of the mitochondrial chaperone Mdj1p in inheritance of mitochondrial DNA in yeast","volume":"19","author":"Duchniewicz","year":"1999","journal-title":"Mol. Cell. Biol"},{"key":"B31","first-page":"505","article-title":"Mitochondrial genetics and functions","volume-title":"The Molecular Biology of the Yeast Saccharomyces: Life Cycle and Inheritance","author":"Dujon","year":"1981"},{"key":"B32","doi-asserted-by":"crossref","first-page":"415","DOI":"10.1093\/genetics\/78.1.415","article-title":"Mitochondrial genetics IX: a model for recombination and segregation of mitochondrial genomes in Saccharomyces cerevisiae","volume":"78","author":"Dujon","year":"1974","journal-title":"Genetics"},{"key":"B33","doi-asserted-by":"publisher","first-page":"403","DOI":"10.1016\/j.dnarep.2003.12.005","article-title":"Repair of oxidative damage in mitochondrial DNA of Saccharomyces cerevisiae: involvement of the MSH1-dependent pathway","volume":"3","author":"Dzierzbicki","year":"2004","journal-title":"DNA Repair (Amst)"},{"key":"B34","doi-asserted-by":"publisher","first-page":"740","DOI":"10.1101\/gad.4.5.740","article-title":"Two genes differentially regulated in the cell cycle and by DNA-damaging agents encode alternative regulatory subunits of ribonucleotide reductase","volume":"4","author":"Elledge","year":"1990","journal-title":"Genes Dev"},{"key":"B35","doi-asserted-by":"publisher","first-page":"1207","DOI":"10.1038\/1761207b0","article-title":"Yeast mitochondria. Subcellular units involved in the synthesis of respiratory enzymes in yeast","volume":"176","author":"Ephrussi","year":"1955","journal-title":"Nature"},{"key":"B36","doi-asserted-by":"publisher","first-page":"18929","DOI":"10.1074\/jbc.270.32.18929","article-title":"Efficient incorporation of anti-HIV deoxynucleotides by recombinant yeast mitochondrial DNA polymerase","volume":"270","author":"Eriksson","year":"1995","journal-title":"J. Biol. Chem"},{"key":"B37","doi-asserted-by":"publisher","first-page":"6376","DOI":"10.1021\/bi200280r","article-title":"Effect of the Y955C mutation on mitochondrial DNA polymerase nucleotide incorporation efficiency and fidelity","volume":"50","author":"Estep","year":"2011","journal-title":"Biochemistry"},{"key":"B38","doi-asserted-by":"publisher","first-page":"9072","DOI":"10.1093\/nar\/gkr618","article-title":"Clustering of Alpers disease mutations and catalytic defects in biochemical variants reveal new features of molecular mechanism of the human mitochondrial replicase, Pol \u00ce\u00b3","volume":"39","author":"Euro","year":"2011","journal-title":"Nucleic Acids Res"},{"key":"B39","doi-asserted-by":"publisher","first-page":"1113","DOI":"10.1016\/j.bbabio.2014.01.021","article-title":"Mapping 136 pathogenic mutations into functional modules in human DNA polymerase \u00ce\u00b3 establishes predictive genotype-phenotype correlations for the complete spectrum of POLG syndromes","volume":"1837","author":"Farnum","year":"2014","journal-title":"Biochim. Biophys. Acta"},{"key":"B40","doi-asserted-by":"crossref","first-page":"20552","DOI":"10.1016\/S0021-9258(19)47098-1","article-title":"Cloning and sequencing of the nuclear gene MIP1 encoding the catalytic subunit of the yeast mitochondrial DNA polymerase","volume":"264","author":"Foury","year":"1989","journal-title":"J. Biol. Chem"},{"key":"B41","doi-asserted-by":"publisher","first-page":"325","DOI":"10.1016\/S0014-5793(98)01467-7","article-title":"The complete sequence of the mitochondrial genome of Saccharomyces cerevisiae","volume":"440","author":"Foury","year":"1998","journal-title":"FEBS Lett"},{"key":"B42","doi-asserted-by":"publisher","first-page":"e27847","DOI":"10.1371\/journal.pone.0027847","article-title":"Antimutator alleles of yeast DNA polymerase gamma modulate the balance between DNA synthesis and excision","volume":"6","author":"Foury","year":"2011","journal-title":"PLoS ONE"},{"key":"B43","doi-asserted-by":"crossref","first-page":"2717","DOI":"10.1002\/j.1460-2075.1992.tb05337.x","article-title":"Yeast mitochondrial DNA mutators with deficient proofreading exonucleolytic activity","volume":"11","author":"Foury","year":"1992","journal-title":"EMBO J"},{"key":"B44","doi-asserted-by":"publisher","first-page":"218","DOI":"10.1038\/283218a0","article-title":"Excision sequences in the mitochondrial genome of yeast","volume":"283","author":"Gaillard","year":"1980","journal-title":"Nature"},{"key":"B45","doi-asserted-by":"crossref","first-page":"9328","DOI":"10.1016\/S0021-9258(18)67658-6","article-title":"A nuclear mutant of Saccharomyces cerevisiae deficient in mitochondrial DNA replication and polymerase activity","volume":"261","author":"Genga","year":"1986","journal-title":"J. Biol. Chem"},{"key":"B46","doi-asserted-by":"publisher","first-page":"851","DOI":"10.1016\/j.molcel.2010.09.002","article-title":"Strand invasion structures in the inverted repeat of Candida albicans mitochondrial DNA reveal a role for homologous recombination in replication","volume":"39","author":"Gerhold","year":"2010","journal-title":"Mol. Cell"},{"key":"B47","doi-asserted-by":"publisher","first-page":"27801","DOI":"10.1074\/jbc.M201756200","article-title":"A bichaperone (Hsp70-Hsp78) system restores mitochondrial DNA synthesis following thermal inactivation of Mip1p polymerase","volume":"277","author":"Germaniuk","year":"2002","journal-title":"J. Biol. Chem"},{"key":"B48","doi-asserted-by":"publisher","first-page":"2729","DOI":"10.1093\/hmg\/ddm227","article-title":"The DNA polymerase gamma Y955C disease variant associated with PEO and parkinsonism mediates the incorporation and translesion synthesis opposite 7,8-dihydro-8-oxo-2\u00e2\u20ac\u00b2-deoxyguanosine","volume":"16","author":"Graziewicz","year":"2007","journal-title":"Hum. Mol. Genet"},{"key":"B49","doi-asserted-by":"publisher","first-page":"770","DOI":"10.1038\/nsmb805","article-title":"Structure-function defects of human mitochondrial DNA polymerase in autosomal dominant progressive external ophthalmoplegia","volume":"11","author":"Graziewicz","year":"2004","journal-title":"Nat. Struct. Mol. Biol"},{"key":"B50","doi-asserted-by":"publisher","first-page":"6973","DOI":"10.1093\/nar\/gkm695","article-title":"A novel mechanism of selectivity against AZT by the human mitochondrial DNA polymerase","volume":"35","author":"Hanes","year":"2007","journal-title":"Nucleic Acids Res"},{"key":"B51","doi-asserted-by":"publisher","first-page":"515","DOI":"10.1016\/S0092-8674(00)80688-1","article-title":"Coupled leading- and lagging-strand synthesis of mammalian mitochondrial DNA","volume":"100","author":"Holt","year":"2000","journal-title":"Cell"},{"key":"B52","doi-asserted-by":"publisher","first-page":"105","DOI":"10.1016\/0378-1119(95)00215-R","article-title":"Isolation and characterization of ten mutator alleles of the mitochondrial DNA polymerase-encoding MIP1 gene from Saccharomyces cerevisiae","volume":"160","author":"Hu","year":"1995","journal-title":"Gene"},{"key":"B53","doi-asserted-by":"publisher","first-page":"40847","DOI":"10.1074\/jbc.M106743200","article-title":"Toxicity of antiviral nucleoside analogs and the human mitochondrial DNA polymerase","volume":"276","author":"Johnson","year":"2001","journal-title":"J. Biol. Chem"},{"key":"B54","doi-asserted-by":"publisher","first-page":"1732","DOI":"10.1016\/j.dnarep.2007.06.005","article-title":"Analysis of Rev1p and Pol zeta in mitochondrial mutagenesis suggests an alternative pathway of damage tolerance","volume":"6","author":"Kalifa","year":"2007","journal-title":"DNA Repair (Amst)"},{"key":"B55","doi-asserted-by":"publisher","first-page":"19501","DOI":"10.1074\/jbc.M109.011940","article-title":"Disease mutations in the human mitochondrial DNA polymerase thumb subdomain impart severe defects in mitochondrial DNA replication","volume":"284","author":"Kasiviswanathan","year":"2009","journal-title":"J. Biol. Chem"},{"key":"B56","doi-asserted-by":"publisher","first-page":"1493","DOI":"10.1517\/17425255.2010.526602","article-title":"Nucleoside reverse transcriptase inhibitor toxicity and mitochondrial DNA","volume":"6","author":"Koczor","year":"2010","journal-title":"Expert. Opin. Drug. Metab. Toxicol"},{"key":"B57","doi-asserted-by":"publisher","first-page":"988","DOI":"10.1007\/s00438-001-0621-x","article-title":"A dominant mitochondrial mutator phenotype of Saccharomyces cerevisiae conferred by msh1 alleles altered in the sequence encoding the ATP-binding domain","volume":"266","author":"Koprowski","year":"2002","journal-title":"Mol. Genet. Genomics"},{"key":"B58","doi-asserted-by":"publisher","first-page":"1861","DOI":"10.1242\/jcs.028605","article-title":"Mitochondrial nucleoids undergo remodeling in response to metabolic cues","volume":"121","author":"Kucej","year":"2008","journal-title":"J. Cell. Sci"},{"key":"B59","doi-asserted-by":"publisher","first-page":"759","DOI":"10.1016\/j.biochi.2012.11.003","article-title":"Biochemical, cellular and molecular identification of DNA polymerase \u00ce\u00b1 in yeast mitochondria","volume":"95","author":"Lasserre","year":"2013","journal-title":"Biochimie"},{"key":"B60","doi-asserted-by":"publisher","first-page":"1","DOI":"10.1007\/BF00290229","article-title":"Overexpression of the RNR1 gene rescues Saccharomyces cerevisiae mutants in the mitochondrial DNA polymerase encoding MIP1 gene","volume":"249","author":"Lecrenier","year":"1995","journal-title":"Mol. Gen. Genet"},{"key":"B61","doi-asserted-by":"publisher","first-page":"37","DOI":"10.1016\/S0378-1119(00)00107-4","article-title":"New features of mitochondrial DNA replication system in yeast and man","volume":"246","author":"Lecrenier","year":"2000","journal-title":"Gene"},{"key":"B62","doi-asserted-by":"publisher","first-page":"14711","DOI":"10.1021\/bi035596s","article-title":"Toxicity of Nucleoside Analogs Used to Treat AIDS and the Selectivity of the Mitochondrial DNA Polymerase","volume":"42","author":"Lee","year":"2003","journal-title":"Biochemistry"},{"key":"B63","doi-asserted-by":"publisher","first-page":"312","DOI":"10.1016\/j.cell.2009.07.050","article-title":"Structural insight into processive human mitochondrial DNA synthesis and disease-related polymerase mutations","volume":"139","author":"Lee","year":"2009","journal-title":"Cell"},{"key":"B64","doi-asserted-by":"publisher","first-page":"141","DOI":"10.1016\/j.bbamcr.2006.01.007","article-title":"Hsp78 chaperone functions in restoration of mitochondrial network following heat stress","volume":"1763","author":"Lewandowska","year":"2006","journal-title":"Biochim. Biophys. Acta"},{"key":"B65","doi-asserted-by":"publisher","first-page":"326","DOI":"10.1038\/labinvest.3700523","article-title":"Decreased mtDNA, oxidative stress, cardiomyopathy, and death from transgenic cardiac targeted human mutant polymerase gamma","volume":"87","author":"Lewis","year":"2007","journal-title":"Lab. Invest"},{"key":"B66","doi-asserted-by":"publisher","first-page":"23616","DOI":"10.1074\/jbc.M101114200","article-title":"Differential incorporation and removal of antiviral deoxynucleotides by human DNA polymerase gamma","volume":"276","author":"Lim","year":"2001","journal-title":"J. Biol. Chem"},{"key":"B67","doi-asserted-by":"publisher","first-page":"38197","DOI":"10.1074\/jbc.274.53.38197","article-title":"The mitochondrial p55 accessory subunit of human DNA polymerase gamma enhances DNA binding, promotes processive DNA synthesis, and confers N-ethylmaleimide resistance","volume":"274","author":"Lim","year":"1999","journal-title":"J. Biol. Chem"},{"key":"B67a","doi-asserted-by":"publisher","first-page":"45","DOI":"10.1016\/S0022-2836(03)00405-4","article-title":"Structural determinants in human DNA polymerase gamma account for mitochondrial toxicity from nucleoside analogs","volume":"23","author":"Lim","year":"2003","journal-title":"J. Mol. Biol"},{"key":"B68","doi-asserted-by":"publisher","first-page":"1133","DOI":"10.1128\/MCB.00770-06","article-title":"DNA recombination-initiation plays a role in the extremely biased inheritance of yeast [rho\u00e2\u02c6\u2019] mitochondrial DNA that contains the replication origin ori5","volume":"27","author":"Ling","year":"2007","journal-title":"Mol. Cell. Biol"},{"key":"B69","doi-asserted-by":"publisher","first-page":"4730","DOI":"10.1093\/emboj\/cdf466","article-title":"Recombination-dependent mtDNA partitioning: in vivo role of Mhr1p to promote pairing of homologous DNA","volume":"21","author":"Ling","year":"2002","journal-title":"EMBO J"},{"key":"B70","doi-asserted-by":"publisher","first-page":"38555","DOI":"10.1074\/jbc.M105230200","article-title":"The fidelity of human DNA polymerase gamma with and without exonucleolytic proofreading and the p55 accessory subunit","volume":"276","author":"Longley","year":"2001","journal-title":"J. Biol. Chem"},{"key":"B71","doi-asserted-by":"publisher","first-page":"13","DOI":"10.1016\/j.mito.2004.04.001","article-title":"Absence of accessory subunit in the DNA polymerase gamma purified from yeast mitochondria","volume":"4","author":"Lucas","year":"2004","journal-title":"Mitochondrion"},{"key":"B72","doi-asserted-by":"crossref","first-page":"3923","DOI":"10.1002\/j.1460-2075.1991.tb04962.x","article-title":"Rolling circle replication of DNA in yeast mitochondria","volume":"10","author":"Maleszka","year":"1991","journal-title":"EMBO J"},{"key":"B73","doi-asserted-by":"publisher","first-page":"308","DOI":"10.1590\/S1415-47572013000300002","article-title":"Replicating animal mitochondrial DNA","volume":"36","author":"McKinney","year":"2013","journal-title":"Genet. Mol. Biol"},{"key":"B74","doi-asserted-by":"publisher","first-page":"503","DOI":"10.1083\/jcb.200304040","article-title":"Evidence for a two membrane-spanning autonomous mitochondrial DNA replisome","volume":"163","author":"Meeusen","year":"2003","journal-title":"J. Cell. Biol"},{"key":"B75","doi-asserted-by":"publisher","first-page":"681","DOI":"10.1146\/annurev.biochem.75.103004.142443","article-title":"Ribonucleotide reductases","volume":"75","author":"Nordlund","year":"2006","journal-title":"Annu. Rev. Biochem"},{"key":"B76","doi-asserted-by":"publisher","first-page":"1115","DOI":"10.1016\/j.jmb.2004.07.096","article-title":"Role of the putative structural protein Sed1p in mitochondrial genome maintenance","volume":"342","author":"Phadnis","year":"2004","journal-title":"J. Mol. Biol"},{"key":"B77","doi-asserted-by":"publisher","first-page":"1549","DOI":"10.1534\/genetics.105.047092","article-title":"Analysis of repeat-mediated deletions in the mitochondrial genome of Saccharomyces cerevisiae","volume":"171","author":"Phadnis","year":"2005","journal-title":"Genetics"},{"key":"B78","doi-asserted-by":"publisher","first-page":"265","DOI":"10.1099\/00221287-90-2-265","article-title":"Manganese mutagenesis in yeast. A practical application of manganese for the induction of mitochondrial antibiotic-resistant mutations","volume":"90","author":"Putrament","year":"1975","journal-title":"J. Gen. Microbiol"},{"key":"B79","doi-asserted-by":"publisher","first-page":"5970","DOI":"10.1074\/jbc.M113.526418","article-title":"Yeast cells expressing the human mitochondrial DNA polymerase reveal correlations between polymerase fidelity and human disease progression","volume":"289","author":"Qian","year":"2014","journal-title":"J. Biol. Chem"},{"key":"B80","doi-asserted-by":"crossref","first-page":"975","DOI":"10.1093\/genetics\/132.4.975","article-title":"Characterization of insertion mutations in the Saccharomyces cerevisiae MSH1 and MSH2 genes: evidence for separate mitochondrial and nuclear functions","volume":"132","author":"Reenan","year":"1992","journal-title":"Genetics"},{"key":"B81","doi-asserted-by":"publisher","first-page":"715","DOI":"10.1016\/0022-2836(73)90291-X","article-title":"Letter: effect of ethidium bromide on transcription of linear and circular DNA templates","volume":"78","author":"Richardson","year":"1973","journal-title":"J. Mol. Biol"},{"key":"B82","doi-asserted-by":"publisher","first-page":"2411","DOI":"10.1093\/hmg\/ddt094","article-title":"Subnormal levels of Pol gamma A cause inefficeint initiation of light-strand DNA synthesis and lead to mitochondrial DNA deletions and autosomal dominant progressive external ophthalmoplegia","volume":"22","author":"Roos","year":"2013","journal-title":"Hum. Mol. Genet"},{"key":"B83","doi-asserted-by":"publisher","first-page":"103","DOI":"10.1016\/0378-1119(95)00412-Y","article-title":"Characterization of a new DNA polymerase from Schizosaccharomyces pombe: a probable homologue of the Saccharomyces cerevisiae DNA polymerase gamma","volume":"165","author":"Ropp","year":"1995","journal-title":"Gene"},{"key":"B84","doi-asserted-by":"publisher","first-page":"449","DOI":"10.1006\/geno.1996.0490","article-title":"Cloning and characterization of the human mitochondrial DNA polymerase, DNA polymerase gamma","volume":"36","author":"Ropp","year":"1996","journal-title":"Genomics"},{"key":"B85","doi-asserted-by":"publisher","first-page":"e322","DOI":"10.1371\/journal.pone.0000322","article-title":"Genome-wide analysis of nucleotide-level variation in commonly used Saccharomyces cerevisiae strains","volume":"2","author":"Schacherer","year":"2007","journal-title":"PLoS ONE"},{"key":"B86","doi-asserted-by":"crossref","first-page":"79","DOI":"10.18388\/abp.1994_4777","article-title":"Characterization of the mitochondrial DNA polymerase from Saccharomyces cerevisiae","volume":"41","author":"Sen","year":"1994","journal-title":"Acta Biochim Pol"},{"key":"B87","doi-asserted-by":"publisher","first-page":"409","DOI":"10.1146\/annurev.biochem.66.1.409","article-title":"Mitochondrial DNA maintenance in vertebrates","volume":"66","author":"Shadel","year":"1997","journal-title":"Annu. Rev. Biochem"},{"key":"B88","doi-asserted-by":"crossref","first-page":"375","DOI":"10.1093\/genetics\/48.3.375","article-title":"Respiration-deficient mutants of yeast. I. Genetics","volume":"48","author":"Sherman","year":"1963","journal-title":"Genetics"},{"key":"B89","doi-asserted-by":"crossref","first-page":"39","DOI":"10.1515\/9783111533391-005","article-title":"Transposable segments of mitochondrial DNA: a unitary hypothesis for the mechanism of mutation, recombination, sequence reiteration and suppressiveness of yeast \u00e2\u20ac\u0153petite colony\u00e2\u20ac\u009d mutants","volume-title":"Mitochondria 1977: Genetics and Biogenesis of Mitochondria","author":"Slonimski","year":"1977"},{"key":"B90","doi-asserted-by":"publisher","first-page":"232","DOI":"10.1016\/0006-291X(68)90440-3","article-title":"Ethidium bromide induced mutation of yeast mitochondria: complete transformation of cells into respiratory deficient non-chromosomal \u00e2\u20ac\u0153petites\u00e2\u20ac\u009d.","volume":"30","author":"Slonimski","year":"1968","journal-title":"Biochem. Biophys. Res. Comm"},{"key":"B91","doi-asserted-by":"publisher","first-page":"8313","DOI":"10.1093\/nar\/12.22.8313","article-title":"Erythromycin and spiramycin resistance mutations of yeast mitochondria: nature of the rib2 locus in the large ribosomal RNA gene","volume":"12","author":"Sor","year":"1984","journal-title":"Nucleic Acids Res"},{"key":"B92","doi-asserted-by":"publisher","first-page":"143","DOI":"10.1007\/s10545-008-1038-z","article-title":"Clinical and molecular features of mitochondrial DNA depletion syndromes","volume":"32","author":"Spinazzola","year":"2009","journal-title":"J. Inherit. Metab. Dis"},{"key":"B93","doi-asserted-by":"publisher","first-page":"1791","DOI":"10.1002\/hep.23891","article-title":"Polymerase \u00ce\u00b3 gene POLG determines the risk of sodium valproate-induced liver toxicity","volume":"52","author":"Stewart","year":"2010","journal-title":"Hepatology"},{"key":"B94","doi-asserted-by":"publisher","first-page":"1181","DOI":"10.1136\/jnnp.2008.166066","article-title":"A variable neurodegenerative phenotype with polymerase gamma mutation","volume":"80","author":"Stricker","year":"2009","journal-title":"J. Neurol. Neurosurg. Psychiatry"},{"key":"B95","doi-asserted-by":"publisher","first-page":"363","DOI":"10.1093\/hmg\/ddi454","article-title":"Mitochondrial and nuclear DNA defects in Saccharomyces cerevisiae with mutations in DNA polymerase gamma associated with progressive external ophthalmoplegia","volume":"15","author":"Stuart","year":"2006","journal-title":"Hum. Mol. Genet"},{"key":"B96","doi-asserted-by":"publisher","first-page":"2123","DOI":"10.1093\/hmg\/ddq089","article-title":"mip1 containing mutations associated with mitochondrial disease causes mutagenesis and depletion of mtDNA in Saccharomyces cerevisiae","volume":"19","author":"Stumpf","year":"2010","journal-title":"Hum. Mol. Genet"},{"key":"B97","doi-asserted-by":"publisher","first-page":"219","DOI":"10.1007\/s00018-010-0530-4","article-title":"Mitochondrial DNA replication and disease: insights from DNA polymerase \u00ce\u00b3 mutations","volume":"68","author":"Stumpf","year":"2011","journal-title":"Cell. Mol. Life. Sci"},{"key":"B98","doi-asserted-by":"publisher","first-page":"519","DOI":"10.1534\/genetics.113.150920","article-title":"The exonuclease activity of the yeast mitochondrial DNA polymerase \u00ce\u00b3 suppresses mitochondrial DNA deletions between short direct repeats in Saccharomyces cerevisiae","volume":"194","author":"Stumpf","year":"2013","journal-title":"Genetics"},{"key":"B99","doi-asserted-by":"publisher","first-page":"e1004748","DOI":"10.1371\/journal.pgen.1004748","article-title":"MMS Exposure Promotes Increased MtDNA Mutagenesis in the Presence of Replication-Defective Disease-Associated DNA Polymerase \u00ce\u00b3 Variants","volume":"10","author":"Stumpf","year":"2014","journal-title":"PLoS Genet"},{"key":"B100","doi-asserted-by":"publisher","first-page":"3516","DOI":"10.1093\/hmg\/ddq267","article-title":"A cluster of pathogenic mutations in the 3\u00e2\u20ac\u00b2-5\u00e2\u20ac\u00b2 exonuclease domain of DNA polymerase gamma defines a novel module coupling DNA synthesis and degradation","volume":"19","author":"Szczepanowska","year":"2010","journal-title":"Hum. Mol. Genet"},{"key":"B101","doi-asserted-by":"publisher","first-page":"248","DOI":"10.1002\/humu.20852","article-title":"Analysis of mutant DNA Polymerase gamma in patients with mitochondrial DNA depletion","volume":"30","author":"Taanman","year":"2008","journal-title":"Hum. Mutat"},{"key":"B102","doi-asserted-by":"publisher","first-page":"417","DOI":"10.1038\/nature02517","article-title":"Premature ageing in mice expressing defective mitochondrial DNA polymerase","volume":"429","author":"Trifunovic","year":"2004","journal-title":"Nature"},{"key":"B103","doi-asserted-by":"publisher","first-page":"23690","DOI":"10.1074\/jbc.273.37.23690","article-title":"The role of 3\u00e2\u20ac\u00b2-5\u00e2\u20ac\u00b2 exonucleolytic proofreading and mismatch repair in yeast mitochondrial DNA error avoidance","volume":"273","author":"Vanderstraeten","year":"1998","journal-title":"J. Biol. Chem"},{"key":"B104","doi-asserted-by":"publisher","first-page":"211","DOI":"10.1038\/90034","article-title":"Mutation of POLG is associated with progressive external ophthalmoplegia characterized by mtDNA deletions","volume":"28","author":"Van Goethem","year":"2001","journal-title":"Nat. Genet"},{"key":"B105","doi-asserted-by":"publisher","first-page":"e33482","DOI":"10.1371\/journal.pone.0033482","article-title":"C-terminal extension of the yeast mitochondrial DNA polymerase determines the balance between synthesis and degradation","volume":"7","author":"Viikov","year":"2012","journal-title":"PLoS ONE"},{"key":"B106","doi-asserted-by":"publisher","first-page":"119","DOI":"10.1016\/j.mito.2010.08.007","article-title":"Yeast mitochondrial DNA polymerase is a highly processive single-subunit enzyme","volume":"11","author":"Viikov","year":"2011","journal-title":"Mitochondrion"},{"key":"B107","doi-asserted-by":"publisher","first-page":"199","DOI":"10.1111\/j.1432-1033.1976.tb10779.x","article-title":"DNA-dependent DNA polymerase from yeast mitochondria. Dependence of enzyme activity on conditions of cell growth, and properties of the highly purified polymerase","volume":"68","author":"Wintersberger","year":"1976","journal-title":"Eur. J. Biochem"},{"key":"B108","doi-asserted-by":"publisher","first-page":"20","DOI":"10.1111\/j.1432-1033.1970.tb00894.x","article-title":"Studies on deoxyribonucleic acid polymerases from yeast. 2. Partial purification and characterization of mitochondrial DNA polymerase from wild type and respiration-deficient yeast cells","volume":"13","author":"Wintersberger","year":"1970","journal-title":"Eur. J. Biochem"},{"key":"B109","doi-asserted-by":"publisher","first-page":"2652","DOI":"10.1158\/0008-5472.CAN-07-5873","article-title":"Broad overexpression of ribonucleotide reductase genes in mice specifically induces lung neoplasms","volume":"68","author":"Xu","year":"2008","journal-title":"Cancer Res"},{"key":"B110","doi-asserted-by":"publisher","first-page":"374","DOI":"10.1074\/jbc.M509730200","article-title":"Functional human mitochondrial DNA polymerase gamma forms a heterotrimer","volume":"281","author":"Yakubovskaya","year":"2006","journal-title":"J. Biol. Chem"},{"key":"B111","doi-asserted-by":"publisher","first-page":"1419","DOI":"10.1086\/513872","article-title":"Novel mutation of human DNA polymerase gamma associated with mitochondrial toxicity induced by anti-HIV treatment","volume":"195","author":"Yamanaka","year":"2007","journal-title":"J. Infect. Dis"},{"key":"B112","doi-asserted-by":"publisher","first-page":"903","DOI":"10.1002\/yea.1644","article-title":"Effects of the S288c genetic background and common auxotrophic markers on mitochondrial DNA function in Saccharomyces cerevisiae","volume":"25","author":"Young","year":"2009","journal-title":"Yeast"},{"key":"B113","doi-asserted-by":"publisher","first-page":"101","DOI":"10.1002\/yea.1344","article-title":"The carboxyl-terminal extension on fungal mitochondrial DNA polymerases: identification of a critical region of the enzyme from Saccharomyces cerevisiae","volume":"23","author":"Young","year":"2006","journal-title":"Yeast"},{"key":"B114","doi-asserted-by":"publisher","first-page":"309","DOI":"10.1038\/339309a0","article-title":"An autosomal dominant disorder with multiple deletions of mitochondrial DNA starting at the D-loop region","volume":"339","author":"Zeviani","year":"1989","journal-title":"Nature"},{"key":"B115","doi-asserted-by":"publisher","first-page":"2683","DOI":"10.1534\/genetics.105.051029","article-title":"Saccharomyces cerevisiae polymerase zeta functions in mitochondria","volume":"172","author":"Zhang","year":"2006","journal-title":"Genetics"},{"key":"B116","doi-asserted-by":"publisher","first-page":"9076","DOI":"10.1128\/MCB.20.23.9076-9083.2000","article-title":"Mutational and structural analyses of the ribonucleotide reductase inhibitor Sml1 define its Rnr1 interaction domain whose inactivation allows suppression of mec1 and rad53 lethality","volume":"20","author":"Zhao","year":"2000","journal-title":"Mol. Cell. Biol"},{"key":"B117","doi-asserted-by":"publisher","first-page":"329","DOI":"10.1016\/S1097-2765(00)80277-4","article-title":"A suppressor of two essential checkpoint genes identifies a novel protein that negatively affects dNTP pools","volume":"2","author":"Zhao","year":"1998","journal-title":"Mol Cell"}],"container-title":["Frontiers in Genetics"],"original-title":[],"deposited":{"date-parts":[[2022,5,3]],"date-time":"2022-05-03T05:01:59Z","timestamp":1651554119000},"score":1,"resource":{"primary":{"URL":"http:\/\/www.frontiersin.org\/Genetic_Disorders\/10.3389\/fgene.2015.00106\/abstract"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2015,3,17]]},"references-count":117,"URL":"https:\/\/doi.org\/10.3389\/fgene.2015.00106","relation":{},"ISSN":["1664-8021"],"issn-type":[{"value":"1664-8021","type":"electronic"}],"subject":[],"published":{"date-parts":[[2015,3,17]]}}}