title: Primer removal during mammalian mitochondrial DNA replication.
abstract: The small circular mitochondrial genome in mammalian cells is replicated by a dedicated replisome, defects in which can cause mitochondrial disease in humans. A fundamental step in mitochondrial DNA (mtDNA) replication and maintenance is the removal of the RNA primers needed for replication initiation. The nucleases RNase H1, FEN1, DNA2, and MGME1 have been implicated in this process. Here we review the role of these nucleases in the light of primer removal pathways in mitochondria, highlight associations with disease, as well as consider the implications for mtDNA replication initiation.
authors: Department of Medical Biochemistry and Cell Biology, University of Gothenburg, PO Box 440, Sweden. Electronic address: jennifer.uhler@medkem.gu.se.
authors: Jay P
authors: JP
authors: Uhler
authors: Department of Medical Biochemistry and Cell Biology, University of Gothenburg, PO Box 440, Sweden. Electronic address: maria.falkenberg@medkem.gu.se.
authors: Maria
authors: M
authors: Falkenberg
mesh_terms: D000818:Animals; D004265:DNA Helicases / Q000378:metabolism; D004261:DNA Replication; D004272:DNA, Mitochondrial / Q000096:biosynthesis*; D005090:Exodeoxyribonucleases / Q000378:metabolism; D045585:Flap Endonucleases / Q000378:metabolism; D054629:Genome, Mitochondrial; D006801:Humans; D028361:Mitochondrial Diseases / Q000201:enzymology; D012313:RNA / Q000378:metabolism*; D016914:Ribonuclease H / Q000378:metabolism
chemical_list: D004272:DNA, Mitochondrial; C034477:RNA primers; D012313:RNA; D005090:Exodeoxyribonucleases; D045585:Flap Endonucleases; D016914:Ribonuclease H; D004265:DNA Helicases
keywords: DNA2; FEN1; MGME1; RNA primer; RNase H1; mtDNA