{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,6]],"date-time":"2026-10-06T06:12:54Z","timestamp":1791267174756,"version":"4.1.0"},"reference-count":38,"publisher":"Oxford University Press (OUP)","issue":"13","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2005,7,1]]},"DOI":"10.1093\/hmg\/ddi184","type":"journal-article","created":{"date-parts":[[2005,5,11]],"date-time":"2005-05-11T20:13:26Z","timestamp":1115842406000},"page":"1775-1783","source":"Crossref","is-referenced-by-count":256,"title":["Mitochondrial DNA polymerase gamma is essential for mammalian embryogenesis"],"prefix":"10.1093","volume":"14","author":[{"given":"Nicole","family":"Hance","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mats I.","family":"Ekstrand","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Aleksandra","family":"Trifunovic","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"286","published-online":{"date-parts":[[2005,5,11]]},"reference":[{"key":"key\n\t\t\t\t20171012152736_DDI184C1","doi-asserted-by":"crossref","unstructured":"Burgers, P.M., Koonin, E.V., Bruford, E., Blanco, L., Burtis, K.C., Christman, M.F., Copeland, W.C., Friedberg, E.C., Hanaoka, F., Hinkle, D.C. et\u00a0al. (2001) Eukaryotic DNA polymerases: proposal for a revised nomenclature. J. Biol. Chem., 276, 43487\u201343490.","DOI":"10.1074\/jbc.R100056200"},{"key":"key\n\t\t\t\t20171012152736_DDI184C2","doi-asserted-by":"crossref","unstructured":"Kaguni, L.S. (2004) DNA polymerase gamma, the mitochondrial replicase. Annu. Rev. Biochem., 73, 293\u2013320.","DOI":"10.1146\/annurev.biochem.72.121801.161455"},{"key":"key\n\t\t\t\t20171012152736_DDI184C3","doi-asserted-by":"crossref","unstructured":"Schultz, R.A., Swoap, S.J., McDaniel, L.D., Zhang, B., Koon, E.C., Garry, D.J., Li, K. and Williams, R.S. (1998) Differential expression of mitochondrial DNA replication factors in mammalian tissues. J. Biol. Chem., 273, 3447\u20133451.","DOI":"10.1074\/jbc.273.6.3447"},{"key":"key\n\t\t\t\t20171012152736_DDI184C4","doi-asserted-by":"crossref","unstructured":"Ye, F., Carrodeguas, J.A. and Bogenhagen, D.F. (1996) The gamma subfamily of DNA polymerases: cloning of a developmentally regulated cDNA encoding Xenopus laevis mitochondrial DNA polymerase gamma. Nucleic Acids Res., 24, 1481\u20131488.","DOI":"10.1093\/nar\/24.8.1481"},{"key":"key\n\t\t\t\t20171012152736_DDI184C5","doi-asserted-by":"crossref","unstructured":"Lecrenier, N., Van Der Bruggen, P. and Foury, F. (1997) Mitochondrial DNA polymerases from yeast to man: a new family of polymerases. Gene, 185, 147\u2013152.","DOI":"10.1016\/S0378-1119(96)00663-4"},{"key":"key\n\t\t\t\t20171012152736_DDI184C6","doi-asserted-by":"crossref","unstructured":"Carrodeguas, J.A., Pinz, K.G. and Bogenhagen, D.F. (2002) DNA binding properties of human pol gammaB. J. Biol. Chem., 277, 50008\u201350014.","DOI":"10.1074\/jbc.M207030200"},{"key":"key\n\t\t\t\t20171012152736_DDI184C7","doi-asserted-by":"crossref","unstructured":"Fan, L. and Kaguni, L.S. (2001) Multiple regions of subunit interaction in Drosophila mitochondrial DNA polymerase: three functional domains in the accessory subunit. Biochemistry, 40, 4780\u20134791.","DOI":"10.1021\/bi010102h"},{"key":"key\n\t\t\t\t20171012152736_DDI184C8","doi-asserted-by":"crossref","unstructured":"Vanderstraeten, S., Van den Brule, S., Hu, J. and Foury, F. (1998) The role of 3\u2032\u20135\u2032 exonucleolytic proofreading and mismatch repair in yeast mitochondrial DNA error avoidance. J. Biol. Chem., 273, 23690\u201323697.","DOI":"10.1074\/jbc.273.37.23690"},{"key":"key\n\t\t\t\t20171012152736_DDI184C9","doi-asserted-by":"crossref","unstructured":"Farr, C.L., Wang, Y. and Kaguni, L.S. (1999) Functional interactions of mitochondrial DNA polymerase and single-stranded DNA-binding protein. Template-primer DNA binding and initiation and elongation of DNA strand synthesis. J. Biol. Chem., 274, 14779\u201314785.","DOI":"10.1074\/jbc.274.21.14779"},{"key":"key\n\t\t\t\t20171012152736_DDI184C10","doi-asserted-by":"crossref","unstructured":"Lim, S.E., Longley, M.J. and Copeland, W.C. (1999) The mitochondrial p55 accessory subunit of human DNA polymerase gamma enhances DNA binding, promotes processive DNA synthesis, and confers N-ethylmaleimide resistance. J. Biol. Chem., 274, 38197\u201338203.","DOI":"10.1074\/jbc.274.53.38197"},{"key":"key\n\t\t\t\t20171012152736_DDI184C11","doi-asserted-by":"crossref","unstructured":"Trifunovic, A., Wredenberg, A., Falkenberg, M., Spelbrink, J.N., Rovio, A.T., Bruder, C.E., Bohlooly, Y.M., Gidlof, S., Oldfors, A., Wibom, R. et\u00a0al. (2004) Premature ageing in mice expressing defective mitochondrial DNA polymerase. Nature, 429, 417\u2013423.","DOI":"10.1038\/nature02517"},{"key":"key\n\t\t\t\t20171012152736_DDI184C12","doi-asserted-by":"crossref","unstructured":"Lewis, W., Simpson, J.F. and Meyer, R.R. (1994) Cardiac mitochondrial DNA polymerase-gamma is inhibited competitively and noncompetitively by phosphorylated zidovudine. Circ. Res., 74, 344\u2013348.","DOI":"10.1161\/01.RES.74.2.344"},{"key":"key\n\t\t\t\t20171012152736_DDI184C13","doi-asserted-by":"crossref","unstructured":"Lewis, W., Day, B.J. and Copeland, W.C. (2003) Mitochondrial toxicity of NRTI antiviral drugs: an integrated cellular perspective. Nat. Rev. Drug. Discov., 2, 812\u2013822.","DOI":"10.1038\/nrd1201"},{"key":"key\n\t\t\t\t20171012152736_DDI184C14","doi-asserted-by":"crossref","unstructured":"Suomalainen, A. and Kaukonen, J. (2001) Diseases caused by nuclear genes affecting mtDNA stability. Am. J. Med. Genet., 106, 53\u201361.","DOI":"10.1002\/ajmg.1379"},{"key":"key\n\t\t\t\t20171012152736_DDI184C15","doi-asserted-by":"crossref","unstructured":"Van Goethem, G., Dermaut, B., Lofgren, A., Martin, J.J. and Van Broeckhoven, C. (2001) Mutation of POLG is associated with progressive external ophthalmoplegia characterized by mtDNA deletions. Nat. Genet., 28, 211\u2013212.","DOI":"10.1038\/90034"},{"key":"key\n\t\t\t\t20171012152736_DDI184C16","doi-asserted-by":"crossref","unstructured":"Lamantea, E., Tiranti, V., Bordoni, A., Toscano, A., Bono, F., Servidei, S., Papadimitriou, A., Spelbrink, H., Silvestri, L., Casari, G. et\u00a0al. (2002) Mutations of mitochondrial DNA polymerase gammaA are a frequent cause of autosomal dominant or recessive progressive external ophthalmoplegia. Ann. Neurol., 52, 211\u2013219.","DOI":"10.1002\/ana.10278"},{"key":"key\n\t\t\t\t20171012152736_DDI184C17","doi-asserted-by":"crossref","unstructured":"Agostino, A., Valletta, L., Chinnery, P.F., Ferrari, G., Carrara, F., Taylor, R.W., Schaefer, A.M., Turnbull, D.M., Tiranti, V. and Zeviani, M. (2003) Mutations of ANT1, Twinkle, and POLG1 in sporadic progressive external ophthalmoplegia (PEO). Neurology, 60, 1354\u20131356.","DOI":"10.1212\/01.WNL.0000056088.09408.3C"},{"key":"key\n\t\t\t\t20171012152736_DDI184C18","doi-asserted-by":"crossref","unstructured":"Van Goethem, G., Schwartz, M., Lofgren, A., Dermaut, B., Van Broeckhoven, C. and Vissing, J. (2003) Novel POLG mutations in progressive external ophthalmoplegia mimicking mitochondrial neurogastrointestinal encephalomyopathy. Eur. J. Hum. Genet., 11, 547\u2013549.","DOI":"10.1038\/sj.ejhg.5201002"},{"key":"key\n\t\t\t\t20171012152736_DDI184C19","doi-asserted-by":"crossref","unstructured":"Naviaux, R.K. and Nguyen, K.V. (2004) POLG mutations associated with Alpers' syndrome and mitochondrial DNA depletion. Ann. Neurol., 55, 706\u2013712.","DOI":"10.1002\/ana.20079"},{"key":"key\n\t\t\t\t20171012152736_DDI184C20","doi-asserted-by":"crossref","unstructured":"Ferrari, G., Lamantea, E., Donati, A., Filosto, M., Briem, E., Carrara, F., Parini, R., Simonati, A., Santer, R. and Zeviani, M. (2005) Infantile hepatocerebral syndromes associated with mutations in the mitochondrial DNA polymerase-gammaA. Brain, 128, 723\u2013731.","DOI":"10.1093\/brain\/awh410"},{"key":"key\n\t\t\t\t20171012152736_DDI184C21","doi-asserted-by":"crossref","unstructured":"Luoma, P., Melberg, A., Rinne, J.O., Kaukonen, J.A., Nupponen, N.N., Chalmers, R.M., Oldfors, A., Rautakorpi, I., Peltonen, L., Majamaa, K. et\u00a0al. (2004) Parkinsonism, premature menopause, and mitochondrial DNA polymerase gamma mutations: clinical and molecular genetic study. Lancet, 364, 875\u2013882.","DOI":"10.1016\/S0140-6736(04)16983-3"},{"key":"key\n\t\t\t\t20171012152736_DDI184C22","doi-asserted-by":"crossref","unstructured":"Rovio, A.T., Marchington, D.R., Donat, S., Schuppe, H.C., Abel, J., Fritsche, E., Elliott, D.J., Laippala, P., Ahola, A.L., McNay, D. et\u00a0al. (2001) Mutations at the mitochondrial DNA polymerase (POLG) locus associated with male infertility. Nat. Genet., 29, 261\u2013262.","DOI":"10.1038\/ng759"},{"key":"key\n\t\t\t\t20171012152736_DDI184C23","doi-asserted-by":"crossref","unstructured":"Ekstrand, M.I., Falkenberg, M., Rantanen, A., Park, C.B., Gaspari, M., Hultenby, K., Rustin, P., Gustafsson, C.M. and Larsson, N.G. (2004) Mitochondrial transcription factor A regulates mtDNA copy number in mammals. Hum. Mol. Genet., 13, 935\u2013944.","DOI":"10.1093\/hmg\/ddh109"},{"key":"key\n\t\t\t\t20171012152736_DDI184C24","doi-asserted-by":"crossref","unstructured":"Larsson, N.G., Wang, J., Wilhelmsson, H., Oldfors, A., Rustin, P., Lewandoski, M., Barsh, G.S. and Clayton, D.A. (1998) Mitochondrial transcription factor A is necessary for mtDNA maintenance and embryogenesis in mice. Nat. Genet., 18, 231\u2013236.","DOI":"10.1038\/ng0398-231"},{"key":"key\n\t\t\t\t20171012152736_DDI184C25","doi-asserted-by":"crossref","unstructured":"Cerritelli, S.M., Frolova, E.G., Feng, C., Grinberg, A., Love, P.E. and Crouch, R.J. (2003) Failure to produce mitochondrial DNA results in embryonic lethality in Rnaseh1 null mice. Mol. Cell, 11, 807\u2013815.","DOI":"10.1016\/S1097-2765(03)00088-1"},{"key":"key\n\t\t\t\t20171012152736_DDI184C26","doi-asserted-by":"crossref","unstructured":"Ebert, K.M., Liem, H. and Hecht, N.B. (1988) Mitochondrial DNA in the mouse preimplantation embryo. J. Reprod. Fertil., 82, 145\u2013149.","DOI":"10.1530\/jrf.0.0820145"},{"key":"key\n\t\t\t\t20171012152736_DDI184C27","doi-asserted-by":"crossref","unstructured":"Piko, L. and Taylor, K.D. (1987) Amounts of mitochondrial DNA and abundance of some mitochondrial gene transcripts in early mouse embryos. Dev. Biol., 123, 364\u2013374.","DOI":"10.1016\/0012-1606(87)90395-2"},{"key":"key\n\t\t\t\t20171012152736_DDI184C28","doi-asserted-by":"crossref","unstructured":"Piko, L. and Matsumoto, L. (1976) Number of mitochondria and some properties of mitochondrial DNA in the mouse egg. Dev. Biol., 49, 1\u201310.","DOI":"10.1016\/0012-1606(76)90253-0"},{"key":"key\n\t\t\t\t20171012152736_DDI184C29","doi-asserted-by":"crossref","unstructured":"Dawid, I.B. and Chase, J.W. (1972) Mitochondrial RNA in Xenopus laevis. II. Molecular weights and other physical properties of mitochondrial ribosomal and 4 s RNA. J. Mol. Biol., 63, 217\u2013231.","DOI":"10.1016\/0022-2836(72)90371-3"},{"key":"key\n\t\t\t\t20171012152736_DDI184C30","doi-asserted-by":"crossref","unstructured":"Chase, J.W. and Dawid, I.B. (1972) Biogenesis of mitochondria during Xenopus laevis development. Dev. Biol., 27, 504\u2013518.","DOI":"10.1016\/0012-1606(72)90189-3"},{"key":"key\n\t\t\t\t20171012152736_DDI184C31","doi-asserted-by":"crossref","unstructured":"Davis, A.F., Ropp, P.A., Clayton, D.A. and Copeland, W.C. (1996) Mitochondrial DNA polymerase gamma is expressed and translated in the absence of mitochondrial DNA maintenance and replication. Nucleic Acids Res., 24, 2753\u20132759.","DOI":"10.1093\/nar\/24.14.2753"},{"key":"key\n\t\t\t\t20171012152736_DDI184C32","doi-asserted-by":"crossref","unstructured":"Wredenberg, A., Wibom, R., Wilhelmsson, H., Graff, C., Wiener, H.H., Burden, S.J., Oldfors, A., Westerblad, H. and Larsson, N.G. (2002) Increased mitochondrial mass in mitochondrial myopathy mice. Proc. Natl Acad. Sci. USA, 99, 15066\u201315071.","DOI":"10.1073\/pnas.232591499"},{"key":"key\n\t\t\t\t20171012152736_DDI184C33","doi-asserted-by":"crossref","unstructured":"Hardt, N., De Kegel, D., Vanheule, L., Villani, G. and Spadari, S. (1980) DNA polymerase gamma, cytochrome c oxidase and mitochondrial integrity in rabbit spleen lymphocytes stimulated with concanavalin A. Exp. Cell. Res., 127, 269\u2013276.","DOI":"10.1016\/0014-4827(80)90432-2"},{"key":"key\n\t\t\t\t20171012152736_DDI184C34","doi-asserted-by":"crossref","unstructured":"Annex, B.H. and Williams, R.S. (1990) Mitochondrial DNA structure and expression in specialized subtypes of mammalian striated muscle. Mol. Cell. Biol., 10, 5671\u20135678.","DOI":"10.1128\/MCB.10.11.5671"},{"key":"key\n\t\t\t\t20171012152736_DDI184C35","doi-asserted-by":"crossref","unstructured":"Virbasius, J.V. and Scarpulla, R.C. (1994) Activation of the human mitochondrial transcription factor A gene by nuclear respiratory factors: a potential regulatory link between nuclear and mitochondrial gene expression in organelle biogenesis. Proc. Natl Acad. Sci. USA, 91, 1309\u20131313.","DOI":"10.1073\/pnas.91.4.1309"},{"key":"key\n\t\t\t\t20171012152736_DDI184C36","doi-asserted-by":"crossref","unstructured":"Scarpulla, R.C. (1997) Nuclear control of respiratory chain expression in mammalian cells. J. Bioenerg. Biomembr., 29, 109\u2013119.","DOI":"10.1023\/A:1022681828846"},{"key":"key\n\t\t\t\t20171012152736_DDI184C37","doi-asserted-by":"crossref","unstructured":"Wu, Z., Puigserver, P., Andersson, U., Zhang, C., Adelmant, G., Mootha, V., Troy, A., Cinti, S., Lowell, B., Scarpulla, R.C. et\u00a0al. (1999) Mechanisms controlling mitochondrial biogenesis and respiration through the thermogenic coactivator PGC-1. Cell, 98, 115\u2013124.","DOI":"10.1016\/S0092-8674(00)80611-X"},{"key":"key\n\t\t\t\t20171012152736_DDI184C38","doi-asserted-by":"crossref","unstructured":"Wang, J., Wilhelmsson, H., Graff, C., Li, H., Oldfors, A., Rustin, P., Bruning, J.C., Kahn, C.R., Clayton, D.A., Barsh, G.S. et\u00a0al. (1999) Dilated cardiomyopathy and atrioventricular conduction blocks induced by heart-specific inactivation of mitochondrial DNA gene expression. Nat. Genet., 21, 133\u2013137.","DOI":"10.1038\/5089"}],"container-title":["Human Molecular Genetics"],"original-title":[],"language":"en","link":[{"URL":"http:\/\/academic.oup.com\/hmg\/article-pdf\/14\/13\/1775\/1717637\/ddi184.pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2017,10,12]],"date-time":"2017-10-12T16:26:58Z","timestamp":1507825618000},"score":1,"resource":{"primary":{"URL":"http:\/\/academic.oup.com\/hmg\/article\/14\/13\/1775\/621050\/Mitochondrial-DNA-polymerase-gamma-is-essential"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2005,5,11]]},"references-count":38,"journal-issue":{"issue":"13","published-online":{"date-parts":[[2005,5,11]]},"published-print":{"date-parts":[[2005,7,1]]}},"URL":"https:\/\/doi.org\/10.1093\/hmg\/ddi184","relation":{},"ISSN":["1460-2083","0964-6906"],"issn-type":[{"value":"1460-2083","type":"electronic"},{"value":"0964-6906","type":"print"}],"subject":[],"published-other":{"date-parts":[[2005,7,1]]},"published":{"date-parts":[[2005,5,11]]}}}