Regulation of Mammalian Mitochondrial Gene Expression: Recent Advances.

TitleRegulation of Mammalian Mitochondrial Gene Expression: Recent Advances.
Publication TypeJournal Article
Year of Publication2017
AuthorsPearce, SF, Rebelo-Guiomar, P, D'Souza, AR, Powell, CA, Van Haute, L, Minczuk, MA
JournalTrends Biochem Sci
Date Published2017 Mar 09

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.

Alternate JournalTrends Biochem. Sci.
Citation Key10.1016/j.tibs.2017.02.003
PubMed ID28285835
Grant ListMC_U105697135 / / Medical Research Council / United Kingdom