Protein trafficking in the mitochondrial intermembrane space: mechanisms and links to human disease

MacPherson, L. and Tokatlidis, K. (2017) Protein trafficking in the mitochondrial intermembrane space: mechanisms and links to human disease. Biochemical Journal, 474(15), pp. 2533-2545. (doi: 10.1042/BCJ20160627) (PMID:28701417) (PMCID:PMC5509380)

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Mitochondria fulfill a diverse range of functions in cells including oxygen metabolism, homeostasis of inorganic ions and execution of apoptosis. Biogenesis of mitochondria relies on protein import pathways that are ensured by dedicated multiprotein translocase complexes localized in all sub-compartments of these organelles. The key components and pathways involved in protein targeting and assembly have been characterized in great detail over the last three decades. This includes the oxidative folding machinery in the intermembrane space, which contributes to the redox-dependent control of proteostasis. Here, we focus on several components of this system and discuss recent evidence suggesting links to human proteopathy.

Item Type:Articles
Glasgow Author(s) Enlighten ID:Tokatlidis, Professor Kostas and MacPherson, Lisa
Authors: MacPherson, L., and Tokatlidis, K.
College/School:College of Medical Veterinary and Life Sciences > Institute of Molecular Cell and Systems Biology
Journal Name:Biochemical Journal
Publisher:Portland Press
ISSN (Online):1470-8728
Copyright Holders:Copyright © 2017 The Authors
First Published:First published in Biochemical Journal 474:2533-2545
Publisher Policy:Reproduced under a Creative Commons License

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Project CodeAward NoProject NamePrincipal InvestigatorFunder's NameFunder RefLead Dept
624931Oxidative Folding and Redox Signalling in the Mitochondria Intermembrane Space.Konstantinos TokatlidisThe Royal Society (ROYSOC)WM120111RI MOLECULAR CELL & SYSTEMS BIOLOGY
743461Characterizing the mitochondrial import and assembly pathway of the malaria parasitesKonstantinos TokatlidisWellcome Trust (WELLCOTR)202308/Z/16/ZRI MOLECULAR CELL & SYSTEMS BIOLOGY