期刊
DNA REPAIR
卷 34, 期 -, 页码 28-38出版社
ELSEVIER SCIENCE BV
DOI: 10.1016/j.dnarep.2015.07.003
关键词
mtDNA; RNA primer; RNase H1; FEN1; DNA2; MGME1
资金
- Swedish Research Council [2013-3621]
- Swedish Cancer Foundation
- ERC Starting Independent Investigator grant (REPMIT)
- Wallenbergs foundation
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, PEN1, 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. (C) 2015 The Authors. Published by Elsevier B.V. This is an open access article under the CC BY-NC-ND license.
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