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William Copeland

Research Profile

Chemicals & Drugs

Physiology

Concepts & Ideas

Genes & Molecular Sequences

Amino Acid Sequences

Anatomy

Mitochondrion

Disorders

Phenomena

Catalysis

Living Beings

Saccharomyces cerevisiae

Co-author Network

Publications

TOP 3
Jeffrey D Stumpf; Russell P Saneto; William C Copeland
Clinical and molecular features of POLG-related mitochondrial disease.
Christal D Sohl; William C Copeland; Rajesh Kasiviswanathan; Karen S Anderson
Mutations in human DNA polymerase γ confer unique mechanisms of catalytic deficiency that mirror the disease severity in mitochondrial disorder patients.
Rajesh Kasiviswanathan; Tammy R L Collins; William C Copeland
The interface of transcription and DNA replication in the mitochondria.

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All Publications

2013: Jeffrey D Stumpf; Russell P Saneto; William C Copeland
Clinical and molecular features of POLG-related mitochondrial disease.
Cold Spring Harbor perspectives in biology 2013;5(4):a011395.
2013: Christal D Sohl; William C Copeland; Rajesh Kasiviswanathan; Karen S Anderson
Mutations in human DNA polymerase γ confer unique mechanisms of catalytic deficiency that mirror the disease severity in mitochondrial disorder patients.
Human molecular genetics 2013;22(6):1074-85.
2012: Rajesh Kasiviswanathan; Tammy R L Collins; William C Copeland
The interface of transcription and DNA replication in the mitochondria.
Biochimica et biophysica acta 2012;1819(9-10):970-8.
2012: Christal D Sohl; Masanori Baba; William C Copeland; Rajesh Kasiviswanathan; Jiae Kim; Hiroaki Mitsuya; Ugo Pradere; Ray F Schinazi; Karen S Anderson
Balancing antiviral potency and host toxicity: identifying a nucleotide inhibitor with an optimal kinetic phenotype for HIV-1 reverse transcriptase.
Molecular pharmacology 2012;82(1):125-33.
2012: Deborah L Croteau; Rajesh Kasiviswanathan; Mahesh Ramamoorthy; Marie L Rossi; Dharmendra Kumar Singh; Peter Sykora; Jane Tian; Zheng Ming Wang; Mansour Akbari; Chandrika Canugovi; William C Copeland; Vilhelm A Bohr
RECQL4 localizes to mitochondria and preserves mitochondrial DNA integrity.
Aging cell 2012;11(3):456-66.
2012: Rajesh Kasiviswanathan; William C Copeland; Margaret A Gustafson; Joel N Meyer
Human mitochondrial DNA polymerase γ exhibits potential for bypass and mutagenesis at UV-induced cyclobutane thymine dimers.
The Journal of biological chemistry 2012;287(12):9222-9.
2012: Christal D Sohl; William C Copeland; Rajesh Kasiviswanathan; Hiroaki Mitsuya; Stefan G Sarafianos; Kamlendra Singh; Karen S Anderson
Mechanism of interaction of human mitochondrial DNA polymerase γ with the novel nucleoside reverse transcriptase inhibitor 4'-ethynyl-2-fluoro-2'-deoxyadenosine indicates a low potential for host toxicity.
Antimicrobial agents and chemotherapy 2012;56(3):1630-4.
2011: Rajesh Kasiviswanathan; William C Copeland
Biochemical analysis of the G517V POLG variant reveals wild-type like activity.
Mitochondrion 2011;11(6):929-34.
2011: Rajesh Kasiviswanathan; William C Copeland
Ribonucleotide discrimination and reverse transcription by the human mitochondrial DNA polymerase.
The Journal of biological chemistry 2011;286(36):31490-500.
2011: Matthew J Young; Rajesh Kasiviswanathan; Fang-Yuan Li; Matthew J Longley; Lee-Jun Wong; William C Copeland
Biochemical analysis of human POLG2 variants associated with mitochondrial disease.
Human molecular genetics 2011;20(15):3052-66.
2011: Jeffrey D Stumpf; William C Copeland
Mitochondrial DNA replication and disease: insights from DNA polymerase γ mutations.
Cellular and molecular life sciences : CMLS 2011;68(2):219-33.
2010: Matthew J Longley; Margaret M Humble; Farida S Sharief; William C Copeland
Disease variants of the human mitochondrial DNA helicase encoded by C10orf2 differentially alter protein stability, nucleotide hydrolysis, and helicase activity.
The Journal of biological chemistry 2010;285(39):29690-702.
2010: William C Copeland
Understanding heterogeneous diseases in mtDNA maintenance.
Methods (San Diego, Calif.) 2010;51(4):363.
2010: Daniel T Shaughnessy; Leroy Worth; Cindy P Lawler; Kimberly A McAllister; Matthew J Longley; William C Copeland
Meeting report: Identification of biomarkers for early detection of mitochondrial dysfunction.
Mitochondrion 2010;10(5):579-81.
2010: Rajesh Kasiviswanathan; Matthew J Longley; Matthew J Young; William C Copeland
Purification and functional characterization of human mitochondrial DNA polymerase gamma harboring disease mutations.
Methods (San Diego, Calif.) 2010;51(4):379-84.
2010: Jeffrey D Stumpf; Christopher M Bailey; Diana Spell; Matthew Stillwagon; Karen S Anderson; William C Copeland
mip1 containing mutations associated with mitochondrial disease causes mutagenesis and depletion of mtDNA in Saccharomyces cerevisiae.
Human molecular genetics 2010;19(11):2123-33.
2010: William C Copeland
The mitochondrial DNA polymerase in health and disease.
Sub-cellular biochemistry 2010;50():211-22.
2009: Sherine Chan; Robert K Naviaux; Alice A Basinger; Kari A Casas; William C Copeland
De novo mutation in POLG leads to haplotype insufficiency and Alpers syndrome.
Mitochondrion 2009;9(5):340-5.
2009: Rajesh Kasiviswanathan; Matthew J Longley; Sherine Chan; William C Copeland
Disease mutations in the human mitochondrial DNA polymerase thumb subdomain impart severe defects in mitochondrial DNA replication.
The Journal of biological chemistry 2009;284(29):19501-10.
2009: Gregory R Stuart; Margaret M Humble; Micheline K Strand; William C Copeland
Transcriptional response to mitochondrial NADH kinase deficiency in Saccharomyces cerevisiae.
Mitochondrion 2009;9(3):211-21.
2009: Sherine Chan; William C Copeland
DNA polymerase gamma and mitochondrial disease: understanding the consequence of POLG mutations.
Biochimica et biophysica acta 2009;1787(5):312-9.
2009: Gregory R Stuart; William C Copeland; Micheline K Strand
Construction and application of a protein and genetic interaction network (yeast interactome).
Nucleic acids research 2009;37(7):e54.
2009: Rajendra Prasad; Matthew J Longley; Farida S Sharief; Esther W Hou; William C Copeland; Samuel H Wilson
Human DNA polymerase theta possesses 5'-dRP lyase activity and functions in single-nucleotide base excision repair in vitro.
Nucleic acids research 2009;37(6):1868-77.
2009: Matthew J Longley; Leslie A Smith; William C Copeland
Preparation of human mitochondrial single-stranded DNA-binding protein.
Methods in molecular biology (Clifton, N.J.) 2009;554():73-85.
2009: Sherine Chan; William C Copeland
Functional analysis of mutant mitochondrial DNA polymerase proteins involved in human disease.
Methods in molecular biology (Clifton, N.J.) 2009;554():59-72.
2009: Salina M Torres; Dale M Walker; Consuelo L McCash; Meghan M Carter; Jessica Ming; Edmund M Cordova; Rachel M Pons; Dennis L Cook; Steven K Seilkop; William C Copeland; Vernon E Walker
Mutational analysis of the mitochondrial tRNA genes and flanking regions in umbilical cord tissue from uninfected infants receiving AZT-based therapies for prophylaxis of HIV-1.
Environmental and molecular mutagenesis 2009;50(1):10-26.
2008: William C Copeland; Matthew J Longley
DNA2 resolves expanding flap in mitochondrial base excision repair.
Molecular cell 2008;32(4):457-8.
2008: Silvio Ferraris; Susanna Clark; Emanuela Garelli; Guido Davidzon; Steven A Moore; Randy Kardon; Rachelle J Bienstock; Matthew J Longley; Michelangelo Mancuso; Purificación Gutiérrez Ríos; Michio Hirano; William C Copeland; Salvatore DiMauro
Progressive external ophthalmoplegia and vision and hearing loss in a patient with mutations in POLG2 and OPA1.
Archives of neurology 2008;65(1):125-31.
2008: William C Copeland
Inherited mitochondrial diseases of DNA replication.
Annual review of medicine 2008;59():131-46.
2007: Maria A Graziewicz; Rachelle J Bienstock; William C Copeland
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.
Human molecular genetics 2007;16(22):2729-39.
2007: William Lewis; Brian Day; James J Kohler; Seyed H Hosseini; Sherine Chan; Elgin C Green; Chad P Haase; Erin S Keebaugh; Robert Long; Tomika Ludaway; Rodney Russ; Jeffrey Steltzer; Nina Tioleco; Robert Santoianni; William C Copeland
Decreased mtDNA, oxidative stress, cardiomyopathy, and death from transgenic cardiac targeted human mutant polymerase gamma.
Laboratory investigation; a journal of technical methods and pathology 2007;87(4):326-35.
2007: Sherine Chan; Janine H Santos; Joel N Meyer; Bhaskar S Mandavilli; Dennis L Cook; Consuelo L McCash; Grace E Kissling; Abraham Nyska; Julie F Foley; Bennett Van Houten; William C Copeland; Vernon E Walker; Kristine L Witt; Jack B Bishop
Mitochondrial toxicity in hearts of CD-1 mice following perinatal exposure to AZT, 3TC, or AZT/3TC in combination.
Environmental and molecular mutagenesis 2007;48(3-4):190-200.
2006: Sherine Chan; Matthew J Longley; William C Copeland
Modulation of the W748S mutation in DNA polymerase gamma by the E1143G polymorphismin mitochondrial disorders.
Human molecular genetics 2006;15(23):3473-83.
2006: Khue V Nguyen; Farida S Sharief; Sherine Chan; William C Copeland; Robert K Naviaux
Molecular diagnosis of Alpers syndrome.
Journal of hepatology 2006;45(1):108-16.
2006: Matthew J Longley; Susanna Clark; Cynthia Yu Wai Man; Gavin Hudson; Steve E Durham; Robert W Taylor; Simon Nightingale; Douglass M Turnbull; William C Copeland; Patrick F Chinnery
Mutant POLG2 disrupts DNA polymerase gamma subunits and causes progressive external ophthalmoplegia.
American journal of human genetics 2006;78(6):1026-34.
2006: William Lewis; James J Kohler; Seyed H Hosseini; Chad P Haase; William C Copeland; Rachelle J Bienstock; Tomika Ludaway; Jamie McNaught; Rodney Russ; Tami Stuart; Robert Santoianni
Antiretroviral nucleosides, deoxynucleotide carrier and mitochondrial DNA: evidence supporting the DNA pol gamma hypothesis.
AIDS (London, England) 2006;20(5):675-84.
2006: Maria A Graziewicz; Matthew J Longley; William C Copeland
DNA polymerase gamma in mitochondrial DNA replication and repair.
Chemical reviews 2006;106(2):383-405.
2006: Gregory R Stuart; Janine H Santos; Micheline K Strand; Bennett Van Houten; William C Copeland
Mitochondrial and nuclear DNA defects in Saccharomyces cerevisiae with mutations in DNA polymerase gamma associated with progressive external ophthalmoplegia.
Human molecular genetics 2006;15(2):363-74.
2005: Sherine Chan; Matthew J Longley; Robert K Naviaux; William C Copeland
Mono-allelic POLG expression resulting from nonsense-mediated decay and alternative splicing in a patient with Alpers syndrome.
DNA repair 2005;4(12):1381-9.
2005: Sherine Chan; Matthew J Longley; William C Copeland
The common A467T mutation in the human mitochondrial DNA polymerase (POLG) compromises catalytic efficiency and interaction with the accessory subunit.
The Journal of biological chemistry 2005;280(36):31341-6.
2005: Matthew J Longley; Maria A Graziewicz; Rachelle J Bienstock; William C Copeland
Consequences of mutations in human DNA polymerase gamma.
Gene 2005;354():125-31.
2005: Shiwei Song; Zachary F Pursell; William C Copeland; Matthew J Longley; Thomas A Kunkel; Christopher K Mathews
DNA precursor asymmetries in mammalian tissue mitochondria and possible contribution to mutagenesis through reduced replication fidelity.
Proceedings of the National Academy of Sciences of the United States of America 2005;102(14):4990-5.
2004: Maria A Graziewicz; Matthew J Longley; Rachelle J Bienstock; Massimo Zeviani; William C Copeland
Structure-function defects of human mitochondrial DNA polymerase in autosomal dominant progressive external ophthalmoplegia.
Nature structural & molecular biology 2004;11(8):770-6.
2004: Rachelle J Bienstock; William C Copeland
Molecular insights into NRTI inhibition and mitochondrial toxicity revealed from a structural model of the human mitochondrial DNA polymerase.
Mitochondrion 2004;4(2-3):203-13.
2004: Maria A Graziewicz; Jane M Sayer; Donald M Jerina; William C Copeland
Nucleotide incorporation by human DNA polymerase gamma opposite benzo[a]pyrene and benzo[c]phenanthrene diol epoxide adducts of deoxyguanosine and deoxyadenosine.
Nucleic acids research 2004;32(1):397-405.
2003: Gopalakrishnan Karthikeyan; Janine H Santos; Maria A Graziewicz; William C Copeland; Grazia Isaya; Bennett Van Houten; Michael A Resnick
Reduction in frataxin causes progressive accumulation of mitochondrial damage.
Human molecular genetics 2003;12(24):3331-42.
2003: William Lewis; Brian Day; William C Copeland
Mitochondrial toxicity of NRTI antiviral drugs: an integrated cellular perspective.
Nature reviews. Drug discovery 2003;2(10):812-22.
2003: Micheline K Strand; Gregory R Stuart; Matthew J Longley; Maria A Graziewicz; Olivia C Dominick; William C Copeland
POS5 gene of Saccharomyces cerevisiae encodes a mitochondrial NADH kinase required for stability of mitochondrial DNA.
Eukaryotic cell 2003;2(4):809-20.
2003: Susan E Lim; Mikhail V Ponamarev; Matthew J Longley; William C Copeland
Structural determinants in human DNA polymerase gamma account for mitochondrial toxicity from nucleoside analogs.
Journal of molecular biology 2003;329(1):45-57.
2003: William C Copeland; Matthew J Longley
DNA polymerase gamma in mitochondrial DNA replication and repair.
TheScientificWorldJournal 2003;3():34-44.
2003: William C Copeland; Mikhail V Ponamarev; Dinh Nguyen; Thomas A Kunkel; Matthew J Longley
Mutations in DNA polymerase gamma cause error prone DNA synthesis in human mitochondrial disorders.
Acta biochimica Polonica 2003;50(1):155-67.
2002: Maria A Graziewicz; Brian Day; William C Copeland
The mitochondrial DNA polymerase as a target of oxidative damage.
Nucleic acids research 2002;30(13):2817-24.
2002: Mikhail V Ponamarev; Matthew J Longley; Dinh Nguyen; Thomas A Kunkel; William C Copeland
Active site mutation in DNA polymerase gamma associated with progressive external ophthalmoplegia causes error-prone DNA synthesis.
The Journal of biological chemistry 2002;277(18):15225-8.
2002: William C Copeland; Joseph T Wachsman; FM Johnson; John S Penta
Mitochondrial DNA alterations in cancer.
Cancer investigation 2002;20(4):557-69.
2002: Matthew J Longley; William C Copeland
Purification, separation, and identification of the human mtDNA polymerase with and without its accessory subunit.
Methods in molecular biology (Clifton, N.J.) 2002;197():245-57.
2002: Micheline K Strand; William C Copeland
Measuring mtDNA mutation rates in Saccharomyces cerevisiae using the mtArg8 assay.
Methods in molecular biology (Clifton, N.J.) 2002;197():151-7.
2001: Peter M J Burgers; E V Koonin; E Bruford; Luis Blanco; K C Burtis; M F Christman; William C Copeland; E C Friedberg; F Hanaoka; DC Hinkle; Christopher W Lawrence; M Nakanishi; H Ohmori; L Prakash; S Prakash; Claude-Agnes Reynaud; A Sugino; T Todo; Z Wang; J C Weill; R Woodgate
Eukaryotic DNA polymerases: proposal for a revised nomenclature.
The Journal of biological chemistry 2001;276(47):43487-90.
2001: Matthew J Longley; Dinh Nguyen; Thomas A Kunkel; William C Copeland
The fidelity of human DNA polymerase gamma with and without exonucleolytic proofreading and the p55 accessory subunit.
The Journal of biological chemistry 2001;276(42):38555-62.
2001: Susan E Lim; William C Copeland
Differential incorporation and removal of antiviral deoxynucleotides by human DNA polymerase gamma.
The Journal of biological chemistry 2001;276(26):23616-23.
2001: William Lewis; William C Copeland; Brian Day
Mitochondrial dna depletion, oxidative stress, and mutation: mechanisms of dysfunction from nucleoside reverse transcriptase inhibitors.
Laboratory investigation; a journal of technical methods and pathology 2001;81(6):777-90.
2001: J S Penta; FM Johnson; Joseph T Wachsman; William C Copeland
Mitochondrial DNA in human malignancy.
Mutation research 2001;488(2):119-33.
1999: Susan E Lim; Matthew J Longley; William C Copeland
The mitochondrial p55 accessory subunit of human DNA polymerase gamma enhances DNA binding, promotes processive DNA synthesis, and confers N-ethylmaleimide resistance.
The Journal of biological chemistry 1999;274(53):38197-203.
1999: Alexandra Vaisman; Susan E Lim; Steve M. Patrick; William C Copeland; DC Hinkle; John Turchi; Stephen G Chaney
Effect of DNA polymerases and high mobility group protein 1 on the carrier ligand specificity for translesion synthesis past platinum-DNA adducts.
Biochemistry 1999;38(34):11026-39.
1999: Farida S Sharief; Patrick J Vojta; P A Ropp; William C Copeland
Cloning and chromosomal mapping of the human DNA polymerase theta (POLQ), the eighth human DNA polymerase.
Genomics 1999;59(1):90-6.
1998: Matthew J Longley; Rajendra Prasad; D K Srivastava; Samuel H Wilson; William C Copeland
Identification of 5'-deoxyribose phosphate lyase activity in human DNA polymerase gamma and its role in mitochondrial base excision repair in vitro.
Proceedings of the National Academy of Sciences of the United States of America 1998;95(21):12244-8.
1998: Matthew J Longley; P A Ropp; Susan E Lim; William C Copeland
Characterization of the native and recombinant catalytic subunit of human DNA polymerase gamma: identification of residues critical for exonuclease activity and dideoxynucleotide sensitivity.
Biochemistry 1998;37(29):10529-39.
1997: William C Copeland
Expression, purification, and characterization of the two human primase subunits and truncated complexes from Escherichia coli.
Protein expression and purification 1997;9(1):1-9.
1996: P A Ropp; William C Copeland
Cloning and characterization of the human mitochondrial DNA polymerase, DNA polymerase gamma.
Genomics 1996;36(3):449-58.
1996: A F Davis; P A Ropp; David A Clayton; William C Copeland
Mitochondrial DNA polymerase gamma is expressed and translated in the absence of mitochondrial DNA maintenance and replication.
Nucleic acids research 1996;24(14):2753-9.
1995: P A Ropp; William C Copeland
Characterization of a new DNA polymerase from Schizosaccharomyces pombe: a probable homologue of the Saccharomyces cerevisiae DNA polymerase gamma.
Gene 1995;165(1):103-7.
1995: William C Copeland; X Tan
Active site mapping of the catalytic mouse primase subunit by alanine scanning mutagenesis.
The Journal of biological chemistry 1995;270(8):3905-13.
1995: Teresa S-F Wang; William C Copeland; L Rogge; Q Dong
Purification of mammalian DNA polymerases: DNA polymerase alpha.
Methods in enzymology 1995;262():77-84.
1995: William C Copeland; Q Dong; Teresa S-F Wang
Rationale for mutagenesis of DNA polymerase active sites: DNA polymerase alpha.
Methods in enzymology 1995;262():294-303.
1993: William C Copeland; Teresa S-F Wang
Enzymatic characterization of the individual mammalian primase subunits reveals a biphasic mechanism for initiation of DNA replication.
The Journal of biological chemistry 1993;268(35):26179-89.
1993: Q Dong; William C Copeland; Teresa S-F Wang
Mutational studies of human DNA polymerase alpha. Serine 867 in the second most conserved region among alpha-like DNA polymerases is involved in primer binding and mispair primer extension.
The Journal of biological chemistry 1993;268(32):24175-82.
1993: Q Dong; William C Copeland; Teresa S-F Wang
Mutational studies of human DNA polymerase alpha. Identification of residues critical for deoxynucleotide binding and misinsertion fidelity of DNA synthesis.
The Journal of biological chemistry 1993;268(32):24163-74.
1993: S Francesconi; William C Copeland; Teresa S-F Wang
In vivo species specificity of DNA polymerase alpha.
Molecular & general genetics : MGG 1993;241(3-4):457-66.
1993: William C Copeland; N K Lam; Teresa S-F Wang
Fidelity studies of the human DNA polymerase alpha. The most conserved region among alpha-like DNA polymerases is responsible for metal-induced infidelity in DNA synthesis.
The Journal of biological chemistry 1993;268(15):11041-9.
1993: William C Copeland; Teresa S-F Wang
Mutational analysis of the human DNA polymerase alpha. The most conserved region in alpha-like DNA polymerases is involved in metal-specific catalysis.
The Journal of biological chemistry 1993;268(15):11028-40.
1993: I Dornreiter; William C Copeland; Teresa S-F Wang
Initiation of simian virus 40 DNA replication requires the interaction of a specific domain of human DNA polymerase alpha with large T antigen.
Molecular and cellular biology 1993;13(2):809-20.
1992: William C Copeland; M S Chen; Teresa S-F Wang
Human DNA polymerases alpha and beta are able to incorporate anti-HIV deoxynucleotides into DNA.
The Journal of biological chemistry 1992;267(30):21459-64.
1991: William C Copeland; Teresa S-F Wang
Catalytic subunit of human DNA polymerase alpha overproduced from baculovirus-infected insect cells. Structural and enzymological characterization.
The Journal of biological chemistry 1991;266(33):22739-48.
1990: K L Hsi; William C Copeland; Teresa S-F Wang
Human DNA polymerase alpha catalytic polypeptide binds ConA and RCA and contains a specific labile site in the N-terminus.
Nucleic acids research 1990;18(21):6231-7.