Ontology highlight
ABSTRACT:
SUBMITTER: Macao B
PROVIDER: S-EPMC4557304 | biostudies-literature | 2015 Jun
REPOSITORIES: biostudies-literature
Macao Bertil B Uhler Jay P JP Siibak Triinu T Zhu Xuefeng X Shi Yonghong Y Sheng Wenwen W Olsson Monica M Stewart James B JB Gustafsson Claes M CM Falkenberg Maria M
Nature communications 20150622
Mitochondrial DNA (mtDNA) polymerase γ (POLγ) harbours a 3'-5' exonuclease proofreading activity. Here we demonstrate that this activity is required for the creation of ligatable ends during mtDNA replication. Exonuclease-deficient POLγ fails to pause on reaching a downstream 5'-end. Instead, the enzyme continues to polymerize into double-stranded DNA, creating an unligatable 5'-flap. Disease-associated mutations can both increase and decrease exonuclease activity and consequently impair DNA lig ...[more]