[HTML][HTML] The fidelity of human DNA polymerase γ with and without exonucleolytic proofreading and the p55 accessory subunit

MJ Longley, D Nguyen, TA Kunkel… - Journal of Biological …, 2001 - ASBMB
Mutations in human mitochondrial DNA influence aging, induce severe neuromuscular
pathologies, cause maternally inherited metabolic diseases, and suppress apoptosis. Since
the genetic stability of mitochondrial DNA depends on the accuracy of DNA polymerase γ
(pol γ), we investigated the fidelity of DNA synthesis by human pol γ. Comparison of the wild-
type 140-kDa catalytic subunit to its exonuclease-deficient derivative indicates pol γ has
high base substitution fidelity that results from high nucleotide selectivity and exonucleolytic …