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Regulation of Mammalian Mitochondrial Gene Expression: Recent Advances

Published version
Peer-reviewed

Type

Article

Change log

Authors

Pearce, SF 
Rebelo-Guiomar, P 
D'Souza, AR 
Powell, CA 
Van Haute, L 

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.

Description

Keywords

Animals, Gene Expression Regulation, Genes, Mitochondrial, Humans, Mitochondria, Mitochondrial Ribosomes, Oxidative Phosphorylation, RNA Processing, Post-Transcriptional

Journal Title

Trends in Biochemical Sciences

Conference Name

Journal ISSN

0968-0004
1362-4326

Volume Title

42

Publisher

Elsevier
Sponsorship
Medical Research Council (MC_U105697135)
Medical Research Council (MC_UU_00015/4)
Medical Research Council (MC_UU_00015/7)