PubRank

William C Copeland

Author PubWeight™ 73.27‹?›

Top papers

Rank Title Journal Year PubWeight™‹?›
1 Mitochondrial toxicity of NRTI antiviral drugs: an integrated cellular perspective. Nat Rev Drug Discov 2003 2.20
2 Decreased mtDNA, oxidative stress, cardiomyopathy, and death from transgenic cardiac targeted human mutant polymerase gamma. Lab Invest 2006 2.14
3 DNA polymerase gamma in mitochondrial DNA replication and repair. Chem Rev 2006 2.12
4 Molecular and clinical genetics of mitochondrial diseases due to POLG mutations. Hum Mutat 2008 2.04
5 Mutant POLG2 disrupts DNA polymerase gamma subunits and causes progressive external ophthalmoplegia. Am J Hum Genet 2006 1.99
6 Structure-function defects of human mitochondrial DNA polymerase in autosomal dominant progressive external ophthalmoplegia. Nat Struct Mol Biol 2004 1.87
7 The common A467T mutation in the human mitochondrial DNA polymerase (POLG) compromises catalytic efficiency and interaction with the accessory subunit. J Biol Chem 2005 1.81
8 DNA precursor asymmetries in mammalian tissue mitochondria and possible contribution to mutagenesis through reduced replication fidelity. Proc Natl Acad Sci U S A 2005 1.67
9 Consequences of mutations in human DNA polymerase gamma. Gene 2005 1.65
10 Site-specific somatic mitochondrial DNA point mutations in patients with thymidine phosphorylase deficiency. J Clin Invest 2003 1.60
11 Mitochondrial DNA replication and disease: insights from DNA polymerase γ mutations. Cell Mol Life Sci 2010 1.58
12 Modulation of the W748S mutation in DNA polymerase gamma by the E1143G polymorphismin mitochondrial disorders. Hum Mol Genet 2006 1.54
13 Long patch base excision repair in mammalian mitochondrial genomes. J Biol Chem 2008 1.44
14 Alpers-Huttenlocher syndrome. Pediatr Neurol 2013 1.44
15 DNA polymerase gamma and mitochondrial disease: understanding the consequence of POLG mutations. Biochim Biophys Acta 2008 1.43
16 Antiretroviral nucleosides, deoxynucleotide carrier and mitochondrial DNA: evidence supporting the DNA pol gamma hypothesis. AIDS 2006 1.43
17 Molecular diagnosis of Alpers syndrome. J Hepatol 2006 1.38
18 The mitochondrial DNA polymerase as a target of oxidative damage. Nucleic Acids Res 2002 1.35
19 Active site mutation in DNA polymerase gamma associated with progressive external ophthalmoplegia causes error-prone DNA synthesis. J Biol Chem 2002 1.34
20 Human DNA polymerase theta possesses 5'-dRP lyase activity and functions in single-nucleotide base excision repair in vitro. Nucleic Acids Res 2009 1.34
21 Structural determinants in human DNA polymerase gamma account for mitochondrial toxicity from nucleoside analogs. J Mol Biol 2003 1.29
22 Reduction in frataxin causes progressive accumulation of mitochondrial damage. Hum Mol Genet 2003 1.24
23 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 2007 1.19
24 Mono-allelic POLG expression resulting from nonsense-mediated decay and alternative splicing in a patient with Alpers syndrome. DNA Repair (Amst) 2005 1.19
25 Oxidative stress-induced apoptosis in neurons correlates with mitochondrial DNA base excision repair pathway imbalance. Nucleic Acids Res 2005 1.19
26 Origins of human mitochondrial point mutations as DNA polymerase gamma-mediated errors. Mutat Res 2006 1.18
27 Mitochondrial and nuclear DNA defects in Saccharomyces cerevisiae with mutations in DNA polymerase gamma associated with progressive external ophthalmoplegia. Hum Mol Genet 2005 1.16
28 POS5 gene of Saccharomyces cerevisiae encodes a mitochondrial NADH kinase required for stability of mitochondrial DNA. Eukaryot Cell 2003 1.14
29 Disease mutations in the human mitochondrial DNA polymerase thumb subdomain impart severe defects in mitochondrial DNA replication. J Biol Chem 2009 1.11
30 Mitochondrial toxicity in hearts of CD-1 mice following perinatal exposure to AZT, 3TC, or AZT/3TC in combination. Environ Mol Mutagen 2007 1.10
31 Biochemical analysis of human POLG2 variants associated with mitochondrial disease. Hum Mol Genet 2011 1.04
32 Progressive external ophthalmoplegia and vision and hearing loss in a patient with mutations in POLG2 and OPA1. Arch Neurol 2008 1.04
33 mip1 containing mutations associated with mitochondrial disease causes mutagenesis and depletion of mtDNA in Saccharomyces cerevisiae. Hum Mol Genet 2010 1.04
34 Mitochondria, energetics, epigenetics, and cellular responses to stress. Environ Health Perspect 2014 1.03
35 Disease variants of the human mitochondrial DNA helicase encoded by C10orf2 differentially alter protein stability, nucleotide hydrolysis, and helicase activity. J Biol Chem 2010 1.00
36 Ribonucleotide discrimination and reverse transcription by the human mitochondrial DNA polymerase. J Biol Chem 2011 1.00
37 RECQL4 localizes to mitochondria and preserves mitochondrial DNA integrity. Aging Cell 2012 0.99
38 R964C mutation of DNA polymerase gamma imparts increased stavudine toxicity by decreasing nucleoside analog discrimination and impairing polymerase activity. Antimicrob Agents Chemother 2009 0.98
39 Purification and functional characterization of human mitochondrial DNA polymerase gamma harboring disease mutations. Methods 2010 0.95
40 Clinical and molecular features of POLG-related mitochondrial disease. Cold Spring Harb Perspect Biol 2013 0.94
41 Human mitochondrial DNA polymerase γ exhibits potential for bypass and mutagenesis at UV-induced cyclobutane thymine dimers. J Biol Chem 2011 0.94
42 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 2004 0.93
43 Biochemical analysis of the G517V POLG variant reveals wild-type like activity. Mitochondrion 2011 0.92
44 Polg2 is essential for mammalian embryogenesis and is required for mtDNA maintenance. Hum Mol Genet 2012 0.91
45 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. Antimicrob Agents Chemother 2011 0.91
46 Balancing antiviral potency and host toxicity: identifying a nucleotide inhibitor with an optimal kinetic phenotype for HIV-1 reverse transcriptase. Mol Pharmacol 2012 0.88
47 The interface of transcription and DNA replication in the mitochondria. Biochim Biophys Acta 2011 0.88
48 A p.R369G POLG2 mutation associated with adPEO and multiple mtDNA deletions causes decreased affinity between polymerase γ subunits. Mitochondrion 2011 0.87
49 Measuring mtDNA mutation rates in Saccharomyces cerevisiae using the mtArg8 assay. Methods Mol Biol 2002 0.84
50 Preparation of human mitochondrial single-stranded DNA-binding protein. Methods Mol Biol 2009 0.84
51 Mutations in human DNA polymerase γ confer unique mechanisms of catalytic deficiency that mirror the disease severity in mitochondrial disorder patients. Hum Mol Genet 2012 0.84
52 Translesion synthesis past acrolein-derived DNA adducts by human mitochondrial DNA polymerase γ. J Biol Chem 2013 0.82
53 Base composition at mtDNA boundaries suggests a DNA triple helix model for human mitochondrial DNA large-scale rearrangements. Mol Genet Metab 2002 0.82
54 Functional analysis of mutant mitochondrial DNA polymerase proteins involved in human disease. Methods Mol Biol 2009 0.82
55 The exonuclease activity of the yeast mitochondrial DNA polymerase γ suppresses mitochondrial DNA deletions between short direct repeats in Saccharomyces cerevisiae. Genetics 2013 0.82
56 A novel POLG gene mutation in 4 children with Alpers-like hepatocerebral syndromes. Arch Neurol 2010 0.81
57 De novo mutation in POLG leads to haplotype insufficiency and Alpers syndrome. Mitochondrion 2009 0.81
58 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. Environ Mol Mutagen 2009 0.81
59 Construction and application of a protein and genetic interaction network (yeast interactome). Nucleic Acids Res 2009 0.81
60 Transcriptional response to mitochondrial NADH kinase deficiency in Saccharomyces cerevisiae. Mitochondrion 2009 0.79
61 Purification, separation, and identification of the human mtDNA polymerase with and without its accessory subunit. Methods Mol Biol 2002 0.79
62 Meeting report: Identification of biomarkers for early detection of mitochondrial dysfunction. Mitochondrion 2010 0.77
63 A novel POLG gene mutation in a patient with SANDO. J Exp Integr Med 2012 0.75
64 Mitochondrial DNA. Methods and protocols. Introduction. Methods Mol Biol 2002 0.75