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Cell and Tissue Research

, Volume 367, Issue 1, pp 5–20

First Online: 14 July 2016Received: 30 May 2016Accepted: 14 June 2016

Abstract

Oxidative phosphorylation OXPHOS is the mechanism whereby ATP, the major energy source for the cell, is produced by harnessing cellular respiration in the mitochondrion. This is facilitated by five multi-subunit complexes housed within the inner mitochondrial membrane. These complexes, with the exception of complex II, are of a dual genetic origin, requiring expression from nuclear and mitochondrial genes. Mitochondrially encoded mRNA is translated on the mitochondrial ribosome mitoribosome and the recent release of the near atomic resolution structure of the mammalian mitoribosome has highlighted its peculiar features. However, whereas some aspects of mitochondrial translation are understood, much is to be learnt about the presentation of mitochondrial mRNA to the mitoribosome, the biogenesis of the machinery, the exact role of the membrane, the constitution of the translocon-insertion machinery and the regulation of translation in the mitochondrion. This review addresses our current knowledge of mammalian mitochondrial gene expression, highlights key questions and indicates how defects in this process can result in profound mitochondrial disease.

KeywordsMitochondria Mitoribosome Mitochondrial translation Protein synthesis Mitochondrial diseases This study was funded by The Wellcome Trust 096919-Z-11-Z and The Barbour Foundation.

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Autor: Nicole Mai - Zofia M. A. Chrzanowska-Lightowlers - Robert N. Lightowlers

Fuente: https://link.springer.com/



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