Copeland, William C.

DNA Polymerase γ in Mitochondrial DNA Replication and Repair

Graziewicz, M. A., Longley, M. J., & Copeland, W. C. (2006) · Chemical Reviews

DNA Repair MechanismsMitochondrial Function and Pathology

Cited by 301

The Fidelity of Human DNA Polymerase γ with and without Exonucleolytic Proofreading and the p55 Accessory Subunit

Longley, M. J., Nguyen, D., Kunkel, T. A., & Copeland, W. C. (2001) · Journal of Biological Chemistry

DNA Repair MechanismsMitochondrial Function and Pathology

Cited by 270

Mutant POLG2 Disrupts DNA Polymerase γ Subunits and Causes Progressive External Ophthalmoplegia

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., & Chinnery, P. F. (2006) · The American Journal of Human Genetics

Mitochondrial Function and PathologyDNA Repair Mechanisms

Cited by 248

The Mitochondrial p55 Accessory Subunit of Human DNA Polymerase γ Enhances DNA Binding, Promotes Processive DNA Synthesis, and Confers N-Ethylmaleimide Resistance

Lim, S. E., Longley, M. J., & Copeland, W. C. (1999) · Journal of Biological Chemistry

DNA Repair MechanismsMitochondrial Function and Pathology

Cited by 226

Role of the mitochondrial DNA replication machinery in mitochondrial DNA mutagenesis, aging and age-related diseases

DeBalsi, K. L., Hoff, K. E., & Copeland, W. C. (2017) · Ageing Research Reviews

Mitochondrial Function and PathologyGenetics, Aging, and Longevity in Model Organisms

Cited by 180

Active Site Mutation in DNA Polymerase γ Associated with Progressive External Ophthalmoplegia Causes Error-prone DNA Synthesis

Ponamarev, M. V., Longley, M. J., Nguyen, D., Kunkel, T. A., & Copeland, W. C. (2002) · Journal of Biological Chemistry

Mitochondrial Function and PathologyDNA Repair Mechanisms

Cited by 156

Origins of human mitochondrial point mutations as DNA polymerase γ-mediated errors

Zheng, W., Khrapko, K., Coller, H. A., Thilly, W. G., & Copeland, W. C. (2006) · Mutation research. Fundamental and molecular mechanisms of mutagenesis

Mitochondrial Function and PathologyDNA Repair Mechanisms

Cited by 141

Structure-function defects of human mitochondrial DNA polymerase in autosomal dominant progressive external ophthalmoplegia

Graziewicz, M. A., Longley, M. J., Bienstock, R. J., Zeviani, M., & Copeland, W. C. (2004) · Nature Structural & Molecular Biology

Mitochondrial Function and PathologyDNA Repair Mechanisms

Cited by 131

Decreased mtDNA, oxidative stress, cardiomyopathy, and death from transgenic cardiac targeted human mutant polymerase γ

Lewis, W., Day, B. J., Kohler, J. J., Hosseini, S. H., Chan, S. S. L., Green, E. C., Haase, C. P., Keebaugh, E. S., Long, R., Ludaway, T., et al. (2007) · Laboratory Investigation

Mitochondrial Function and PathologyCardiac electrophysiology and arrhythmias

Cited by 121

Polg2 is essential for mammalian embryogenesis and is required for mtDNA maintenance

Humble, M. M., Young, M. J., Foley, J. F., Pandiri, A. R., Travlos, G. S., & Copeland, W. C. (2013) · Human Molecular Genetics

Mitochondrial Function and PathologyDNA Repair Mechanisms

Cited by 78

Ribonucleotide Discrimination and Reverse Transcription by the Human Mitochondrial DNA Polymerase

Kasiviswanathan, R., & Copeland, W. C. (2011) · Journal of Biological Chemistry

DNA and Nucleic Acid ChemistryDNA Repair Mechanisms

Cited by 71

Biochemical analysis of human POLG2 variants associated with mitochondrial disease

Young, M. J., Longley, M. J., Li, F.-Y., Kasiviswanathan, R., Wong, L.-J., & Copeland, W. C. (2011) · Human Molecular Genetics

Mitochondrial Function and PathologyDNA Repair Mechanisms

Cited by 66

POLG2 disease variants: analyses reveal a dominant negative heterodimer, altered mitochondrial localization and impaired respiratory capacity

Young, M. J., Humble, M. M., DeBalsi, K. L., Sun, K. Y., & Copeland, W. C. (2015) · Human Molecular Genetics

Mitochondrial Function and PathologyDNA Repair Mechanisms

Cited by 32

A p.R369G POLG2 mutation associated with adPEO and multiple mtDNA deletions causes decreased affinity between polymerase γ subunits

Craig, K., Young, M. J., Blakely, E. L., Longley, M. J., Turnbull, D. M., Copeland, W. C., & Taylor, R. W. (2012) · Mitochondrion

Mitochondrial Function and PathologyDNA Repair Mechanisms

Cited by 24