PMID- 28285835 OWN - NLM STAT- MEDLINE DCOM- 20171011 LR - 20210109 IS - 0968-0004 (Print) IS - 0968-0004 (Linking) VI - 42 IP - 8 DP - 2017 Aug TI - Regulation of Mammalian Mitochondrial Gene Expression: Recent Advances. PG - 625-639 LID - S0968-0004(17)30042-7 [pii] LID - 10.1016/j.tibs.2017.02.003 [doi] AB - Perturbation of mitochondrial DNA (mtDNA) gene expression can lead to human pathologies. Therefore, a greater appreciation of the basic mechanisms of mitochondrial gene expression is desirable to understand the pathophysiology of associated disorders. Although the purpose of the mitochondrial gene expression machinery is to provide only 13 proteins of the oxidative phosphorylation (OxPhos) system, recent studies have revealed its remarkable and unexpected complexity. We review here the latest breakthroughs in our understanding of the post-transcriptional processes of mitochondrial gene expression, focusing on advances in analyzing the mitochondrial epitranscriptome, the role of mitochondrial RNA granules (MRGs), the benefits of recently obtained structures of the mitochondrial ribosome, and the coordination of mitochondrial and cytosolic translation to orchestrate the biogenesis of OxPhos complexes. CI - Crown Copyright (c) 2017. Published by Elsevier Ltd. All rights reserved. FAU - Pearce, Sarah F AU - Pearce SF AD - Mitochondrial Genetics, Medical Research Council (MRC) Mitochondrial Biology Unit, Hills Road, Cambridge CB2 0XY, UK. FAU - Rebelo-Guiomar, Pedro AU - Rebelo-Guiomar P AD - Mitochondrial Genetics, Medical Research Council (MRC) Mitochondrial Biology Unit, Hills Road, Cambridge CB2 0XY, UK; Graduate Program in Areas of Basic and Applied Biology (GABBA), University of Porto, Porto, Portugal. FAU - D'Souza, Aaron R AU - D'Souza AR AD - Mitochondrial Genetics, Medical Research Council (MRC) Mitochondrial Biology Unit, Hills Road, Cambridge CB2 0XY, UK. FAU - Powell, Christopher A AU - Powell CA AD - Mitochondrial Genetics, Medical Research Council (MRC) Mitochondrial Biology Unit, Hills Road, Cambridge CB2 0XY, UK. FAU - Van Haute, Lindsey AU - Van Haute L AD - Mitochondrial Genetics, Medical Research Council (MRC) Mitochondrial Biology Unit, Hills Road, Cambridge CB2 0XY, UK. FAU - Minczuk, Michal AU - Minczuk M AD - Mitochondrial Genetics, Medical Research Council (MRC) Mitochondrial Biology Unit, Hills Road, Cambridge CB2 0XY, UK. Electronic address: michal.minczuk@mrc-mbu.cam.ac.uk. LA - eng GR - MC_U105697135/MRC_/Medical Research Council/United Kingdom GR - MC_UU_00015/4/MRC_/Medical Research Council/United Kingdom PT - Journal Article PT - Review DEP - 20170309 PL - England TA - Trends Biochem Sci JT - Trends in biochemical sciences JID - 7610674 SB - IM MH - Animals MH - Gene Expression Regulation/*genetics MH - Genes, Mitochondrial/*genetics MH - Humans MH - Mitochondria/*genetics/metabolism MH - Mitochondrial Ribosomes/chemistry/*metabolism MH - *Oxidative Phosphorylation MH - RNA Processing, Post-Transcriptional/genetics PMC - PMC5538620 EDAT- 2017/03/14 06:00 MHDA- 2017/10/12 06:00 PMCR- 2017/08/01 CRDT- 2017/03/14 06:00 PHST- 2016/11/18 00:00 [received] PHST- 2017/02/02 00:00 [revised] PHST- 2017/02/10 00:00 [accepted] PHST- 2017/03/14 06:00 [pubmed] PHST- 2017/10/12 06:00 [medline] PHST- 2017/03/14 06:00 [entrez] PHST- 2017/08/01 00:00 [pmc-release] AID - S0968-0004(17)30042-7 [pii] AID - 10.1016/j.tibs.2017.02.003 [doi] PST - ppublish SO - Trends Biochem Sci. 2017 Aug;42(8):625-639. doi: 10.1016/j.tibs.2017.02.003. Epub 2017 Mar 9.