title: Regulation of Mammalian Mitochondrial Gene Expression: Recent Advances.
abstract: 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.
authors.affiliation: Mitochondrial Genetics, Medical Research Council (MRC) Mitochondrial Biology Unit, Hills Road, Cambridge CB2 0XY, UK.
authors.forename: Sarah F
authors.initials: SF
authors.lastname: Pearce
authors.affiliation: 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.
authors.forename: Pedro
authors.initials: P
authors.lastname: Rebelo-Guiomar
authors.affiliation: Mitochondrial Genetics, Medical Research Council (MRC) Mitochondrial Biology Unit, Hills Road, Cambridge CB2 0XY, UK.
authors.forename: Aaron R
authors.initials: AR
authors.lastname: D'Souza
authors.affiliation: Mitochondrial Genetics, Medical Research Council (MRC) Mitochondrial Biology Unit, Hills Road, Cambridge CB2 0XY, UK.
authors.forename: Christopher A
authors.initials: CA
authors.lastname: Powell
authors.affiliation: Mitochondrial Genetics, Medical Research Council (MRC) Mitochondrial Biology Unit, Hills Road, Cambridge CB2 0XY, UK.
authors.forename: Lindsey
authors.initials: L
authors.lastname: Van Haute
authors.affiliation: Mitochondrial Genetics, Medical Research Council (MRC) Mitochondrial Biology Unit, Hills Road, Cambridge CB2 0XY, UK. Electronic address: michal.minczuk@mrc-mbu.cam.ac.uk.
authors.forename: Michal
authors.initials: M
authors.lastname: Minczuk
mesh_terms: D000818:Animals; D005786:Gene Expression Regulation / Q000235:genetics*; D050259:Genes, Mitochondrial / Q000235:genetics*; D006801:Humans; D008928:Mitochondria / Q000235:genetics* / Q000378:metabolism; D000069396:Mitochondrial Ribosomes / Q000737:chemistry / Q000378:metabolism*; D010085:Oxidative Phosphorylation; D012323:RNA Processing, Post-Transcriptional / Q000235:genetics