Kasiviswanathan, R., Longley, M.J., Chan, S.S.L., and Copeland, W.C. (2009) Disease mutations in the human mitochondrial DNA polymerase thumb subdomain impart severe defects in mtDNA replication. J. Biol. Chem. 284(29): 19501-19510. [Abstract] (http://www.ncbi.nlm.nih.gov/pubmed/19478085?ordinalpos=1&itool=EntrezSystem2.PEntrez.Pubmed.Pubmed_ResultsPanel.Pubmed_DefaultReportPanel.Pubmed_RVDocSum)
Chan, S.S.L., and Copeland, W.C. Functional analysis of mutant mitochondrial DNA polymerase involved in disease. In Mitochondrial DNA: Methods and Protocols, 2nd Edition. (J. Stuart, ed) Human Press, July 22, 2009. 554: 59-72. [Abstract] (http://www.ncbi.nlm.nih.gov/pubmed/19513667?ordinalpos=3&itool=EntrezSystem2.PEntrez.Pubmed.Pubmed_ResultsPanel.Pubmed_DefaultReportPanel.Pubmed_RVDocSum)
Longley, M.J., Smith, L.A., and Copeland, W.C. Preparation of human mitochondrial single stranded DNA binding protein. In Mitochondrial DNA: Methods and Protocols, 2nd Edition. (J. Stuart, ed) Humana Press, July 22, 2009. 554: 73-85. [Abstract](http://www.ncbi.nlm.nih.gov/pubmed/19513668?ordinalpos=1&itool=EntrezSystem2.PEntrez.Pubmed.Pubmed_ResultsPanel.Pubmed_DefaultReportPanel.Pubmed_RVDocSum)
Chan, S.S.L., Naviaux, R.K., Basinger, A.A., Casas, K.A., and Copeland, W.C. (2009) DeNovo Mutation in POLG Leads to Haplotype Insufficiency and Alpers Syndrome. Mitochondrion. (in press) 2009 Jun 6. [Epub ahead of print]. [Abstract](http://www.ncbi.nlm.nih.gov/pubmed/19501198?ordinalpos=2&itool=EntrezSystem2.PEntrez.Pubmed.Pubmed_ResultsPanel.Pubmed_DefaultReportPanel.Pubmed_RVDocSum)
Prasad, R., Longley, M.J., Sharief, F.S., Hou, E.W., Copeland, W.C., and Wilson, S.H. (2009) Human DNA polymerase theta possesses 5'-dRP lyase activity and functions in single-nucleotide base excision repair in vitro. Nucleic Acids Res. 37(6): 1868-1877. [Abstract](http://www.ncbi.nlm.nih.gov/pubmed/19188258?ordinalpos=3&itool=EntrezSystem2.PEntrez.Pubmed.Pubmed_ResultsPanel.Pubmed_DefaultReportPanel.Pubmed_RVDocSum)
Stuart, G.R., Humble, M.H., Strand, M.K., and Copeland, W.C. (2009) Transcriptional response to mitochondrial NADH kinase deficiency in saccharomyces cerevisiae. Mitochondrion 9(3):211-221. [Abstract](http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=Abstract&list_uids=19254780)
Bailey, C.M., Kasiviswanathan, K., Copeland, W.C., and Anderson, K.S. (2009) R964C mutation of DNA polymerase³ imparts increased stavudine toxicity by decreasing nucleoside analog discrimination and impairing polymerase activity. Antimicrobial agents and chemotherapy 53(6):2610-2612. [Abstract](http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=Abstract&list_uids=19364868)
Chan, S.S.L., and Copeland, W.C. (2009) DNA polymerase gamma and mitochondrial disease: understanding the consequence of POLG mutations. BBA - Bioenergetics 1676(5):312-319. [Abstract](http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=Abstract&list_uids=19010300)
Stuart, G.R., Copeland, W.C., and Strand, M.K. (2009) Construction and application of a protein and genetic interaction network (Yeast Interactome). Nucleic acids research 37: e54. [Abstract](http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=Abstract&list_uids=19273534)
Szczesny, B., Tann, A.W., Longley, M.J., Copeland, W.C., and Mitra, S. (2008) Long patch base excision repair in mammalian mitochondrial genomes. The Journal of biological chemistry 283(39):26349-26356. [Abstract](http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=Abstract&list_uids=18635552)
Wong, L.J., Naviaux, R.K., Brunetti-Pierri, N., Zhang, Q., Schmitt, E.S., Truong, C., Milone, M., Cohen, B.H., Wical, B., Ganesh, J., Basinger, A.A., Burton, B.K., Swoboda, K., Gilbert, D.L., Vanderver, A., Saneto, R.P., Maranda, B., Arnold, G., Abdenur, J.E., Waters, P.J., and Copeland, W.C. (2008) Molecular and clinical genetics of mitochondrial diseases due to POLG mutations. Human mutation 29(9):E150-E172. [Abstract](http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=Abstract&list_uids=18546365)
Ferraris, S., Clark, S., Garelli, E., Davidzon, G., Moore, S.A., Kardon, R.H., Bienstock, R.J., Longley, M.J., Mancuso, M., Rios, P.G., Hirano, M., Copeland, W.C., and DiMauro, S. (2008) Progressive external ophthalmoplegia, vision, and hearing loss in a patient with mutations in POLG2 and OPA1. Archives of neurology 65(1):125-131. [Abstract](http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=Abstract&list_uids=18195150)
Copeland, W.C. (2008) Inherited mitochondrial diseases of DNA Replication.Annu. Rev. Med. 59: 131-146. [Abstract] (http://www.ncbi.nlm.nih.gov/sites/entrez?Db=pubmed&Cmd=ShowDetailView&TermToSearch=17892433&ordinalpos=1&itool=EntrezSystem2.PEntrez.Pubmed.Pubmed_ResultsPanel.Pubmed_RVDocSum)
Copeland, W.C., and Longley, M.J. (2008) DNA2 resolves expanding flap in mitochondrial base excision repair. Mol. Cell. 32, 457-458 [Abstract] (http://www.ncbi.nlm.nih.gov/pubmed/19026774?ordinalpos=11&itool=EntrezSystem2.PEntrez.Pubmed.Pubmed_ResultsPanel.Pubmed_DefaultReportPanel.Pubmed_RVDocSum)
Graziewicz, M.A., Bienstock, R.J., and Copeland, W.C. (2007) 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'-deoxyguanosine. Hum. Mol. Genet. 16:2729–2739. [Abstract] (http://www.ncbi.nlm.nih.gov/sites/entrez?Db=pubmed&Cmd=ShowDetailView&TermToSearch=17725985&ordinalpos=1&itool=EntrezSystem2.PEntrez.Pubmed.Pubmed_ResultsPanel.Pubmed_RVDocSum)
Lewis, W., Day, B.J., Kohler, J.J., Hosseini, S.H., Chan, S.S.L., Green, E., Haase, C.P., Keebaugh, E., Long, R., Ludaway, T., Russ, R., Steltzer, J., Tioleco, N., Santoianni, R., and Copeland, W.C. (2007) Decreased MtDNA, Oxidative Stress, Cardiomyopathy, and Death from Transgenic Cardiac Targeted Human Mutant Polymerase γ. Laboratory Investigation. 87: 326-335. [Abstract]http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?db=pubmed&cmd=Retrieve&dopt=AbstractPlus&list_uids=17310215&query_hl=2&itool=pubmed_docsum
Chan, S.S.L., Santos, J.H., Meyer, J.N., Mandavilli, B.S., Cook, Jr., D.L., McCash, C.L., Van Houten, B., Copeland, W.C., Walker, V.E., and Bishop, J.B. (2007) Mitochondrial toxicity following perinatal exposure of CD-1 mice to AZT, 3TC, or AZT-3TC. Env. Mol. Mut. 48:190-200. [Abstract]http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?db=pubmed&cmd=Retrieve&dopt=AbstractPlus&list_uids=16395692&query_hl=4&itool=pubmed_docsum
Chan, S.S.L., Longley, M.J., and Copeland, W.C. (2006) Modulation of the W748S mutation in DNA polymerase γ by the single nucleotide polymorphism, E1143G, in mitochondrial disorders. Hum Mol Genetics 15(23): 3473-3483. [Abstract]http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?db=pubmed&cmd=Retrieve&dopt=AbstractPlus&list_uids=17088268&query_hl=11&itool=pubmed_docsum
Graziewicz, M.A., Longley, M.J., and Copeland, W.C. (2006) DNA polymerase gamma in mitochondrial DNA replication and repair. Chemical Reviews (Ed. Robert Kuchta) 106(2): 383-405. [Abstract]http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=Abstract&list_uids=16464011
Longley, M.J., Clark, S., Yu Wai Man, C., Hudson, G., Durham, S.E., Taylor, R.W., Nightingale, S., Turnbull, D.M., Copeland, W.C., and Chinnery, P.F. (2006) Mutant POLG2 disrupts DNA polymerase gamma subunits and causes progressive external ophthalmoplegia. Am. J. Hum. Genet. 78(6): 1026-1034. [Abstract]http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=Abstract&list_uids=16685652
Lewis, W., Kohler, J.J., Hosseini, S.H., Haase, C.P., Copeland. W.C., Bienstock, R.J., Ludaway, T., McNaught, J., Russ, R., Stuart, T., and Santoianni, R. (2006) Antiretroviral nucleosides, deoxynucleotide carrier, and mitochondrial DNA: evidence supporting the DNA pol gamma hypothesis. AIDS, 20(5): 675-684. [Abstract]http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?db=pubmed&cmd=Retrieve&dopt=AbstractPlus&list_uids=16514297&query_hl=16&itool=pubmed_docsum
Stuart, G.R., Santos, J.H., Strand, M.K., Van Houten, B., and Copeland, W.C. (2006) Mitochondrial and Nuclear DNA Defects in Saccharomyces cerevisiae With Mutations in DNA Polymerase gamma Associated With Progressive External Ophthalmoplegia Hum. Mol. Genetics 15(2): 363-374. [Abstract]http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=Abstract&list_uids=16368709
Nguyen, K.V., Sharief, F.S., Chan, S.S.L., Copeland, W.C. and Naviaux, R.K. (2006) Molecular diagnosis of Alpers Syndrome. J. of Hepatology 45(1): 108-116. [Abstract]http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=Abstract&list_uids=16545482
Zheng, W., Khrapko, K., Coller, H.A., Thilly, W.G., and Copeland, W.C. (2006) Origins of human mitochondrial point mutations as DNA polymerase gamma–mediated errors. Mutation Research 599(1-2): 11-20. [Abstract]http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=Abstract&list_uids=16490220
Chan, S.S.L., Longley, M.J., and Copeland, W.C. (2005) The common A467T mutation in the human mitochondrial DNA polymerase (POLG) compromises catalytic efficiency and interaction with the accessory subunit. J. Biol. Chem. 280(36): 31341-31346. [Abstract]http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=Abstract&list_uids=16024923
Chan, S.S.L., Longley, M.J., Naviaux, R.K., and Copeland, W.C. (2005) Mono-allelic POLG expression resulting from nonsense-mediated decay and alternative splicing in a patient with Alpers syndrome. DNA Repair. 4(12): 1381-1389. [Abstract]http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=Abstract&list_uids=16181814
Longley, M.J., Graziewicz, M.A., Bienstock, R.J., and Copeland, W.C. (2005) Consequences of mutations in human DNA polymerase gamma. Gene 354: 125-131. [Abstract]http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=Abstract&list_uids=15913923
Song, S., Pursell, Z.F., Copeland, W.C., Longley, M.J., Kunkel, T.A., and Mathews, C.K. (2005) DNA precursor asymmetries in mammalian mitochondrial tissues and possible contribution to mitochondrial mutagenesis through reduced replication fidelity. Proc. Natl. Acad. Sci. USA. 102(14): 4990-4995. [Abstract]http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=Abstract&list_uids=15784738
Graziewicz, M.A., Longley, M.J., Bienstock, R.J., Zeviani, M., and Copeland W.C. (2004) Structure-function defects of human mitochondrial DNA polymerase in autosomal dominant progressive external ophthalmoplegia. Nat. Struct. Mol. Biol. 11(8): 770-776. [Abstract]http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=Abstract&list_uids=15258572
Bienstock, R.J., and Copeland, W.C. (2004) Molecular insights into NRTI inhibition and mitochondrial toxicity revealed from a structural model of the human mitochondrial DNA polymerase. Mitochondrion 4(2-3): 203-213. [Abstract]http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=Abstract&list_uids=16120386
Graziewicz, M.A., Sayer, J.M., Jerina, D.J., and Copeland, W.C. (2004) Nucleotide incorporation by human DNA polymerase gamma opposite benzo[a]pyrene and benzo[c]phenanthrene diol epoxide adducts of deoxyguanosine and deoxyadenosine. Nucleic Acids Res. 32(1): 397-405. [Abstract]http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=Abstract&list_uids=14729924
Strand, M.K., Stuart, G., Longley, M.J., Graziewicz, M.A., Dominick, O.C., and Copeland, W.C. (2003) POS5 gene of Saccharomyces cerevisiae encodes a mitochondrial NADH kinase required for stability of mitochondrial DNA. Eukaryotic Cell 2(4): 809-820. [Abstract]http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=Abstract&list_uids=12912900
Lim, S.E., Ponamarev, M.V., Longley, M.J., and Copeland, W.C. (2003) Structural determinants in human DNA polymerase gamma account for mitochondrial toxicity from nucleoside analogs J. Mol. Biol. 329(1): 45-57. [Abstract]http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=Abstract&list_uids=12742017
Ponamarev, M.V., Longley, M.J., Nguyen, D., Kunkel, T.A., and Copeland, W.C. (2002) Active site mutation in DNA polymerase gamma associated with progressive external ophthalmoplegia causes error-prone DNA synthesis. J. Biol. Chem. 277(18): 15225-15228. [Abstract]http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=Abstract&list_uids=11897778
Graziewicz, M.A., Day, B.J., and Copeland, W.C. (2002) The mitochondrial DNA polymerase as a target of oxidative damage. Nucleic Acids Res. 30(13): 2817-2824. [Abstract]http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=Abstract&list_uids=12087165
Copeland, W.C., Editor (2002) Mitochondrial DNA: Methods and Protocols. Humana Press. Vol. 197. 432 pp. [Abstract]http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=Abstract&list_uids=12013816
Longley, M.J., Nguyen, D., Kunkel, T.A., and Copeland, W.C. (2001) The fidelity of human DNA polymerase gamma with and without exonucleolytic proofreading and the p55 accessory subunit. J. Biol. Chem. 276(42): 38555–38562. [Abstract]http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?db=pubmed&cmd=Retrieve&dopt=AbstractPlus&list_uids=11504725&query_hl=22&itool=pubmed_docsum
Lim, S.E., and Copeland, W.C. (2001) Differential incorporation and removal of antiviral deoxynucleotides by human DNA polymerase gamma. J. Biol. Chem. 276(26): 23616-23623. [Abstract]http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=Abstract&list_uids=11319228
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