Protein import in mitochondria biogenesis: Guided by targeting signals and sustained by dedicated chaperones

Dimogkioka, A.-R., Lees, J., Tokatlidis, K. and Lacko, E. (2021) Protein import in mitochondria biogenesis: Guided by targeting signals and sustained by dedicated chaperones. RSC Advances, 11(51), pp. 32476-32493. (doi: 10.1039/D1RA04497D)

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Abstract

Mitochondria have a central role in cellular metabolism; they are responsible for the biosynthesis of amino acids, lipids, iron–sulphur clusters and regulate apoptosis. About 99% of mitochondrial proteins are encoded by nuclear genes, so the biogenesis of mitochondria heavily depends on protein import pathways into the organelle. An intricate system of well-studied import machinery facilitates the import of mitochondrial proteins. In addition, folding of the newly synthesized proteins takes place in a busy environment. A system of folding helper proteins, molecular chaperones and co-chaperones, are present to maintain proper conformation and thus avoid protein aggregation and premature damage. The components of the import machinery are well characterised, but the targeting signals and how they are recognised and decoded remains in some cases unclear. Here we provide some detail on the types of targeting signals involved in the protein import process. Furthermore, we discuss the very elaborate chaperone systems of the intermembrane space that are needed to overcome the particular challenges for the folding process in this compartment. The mechanisms that sustain productive folding in the face of aggregation and damage in mitochondria are critical components of the stress response and play an important role in cell homeostasis.

Item Type:Articles
Additional Information:Work in our laboratory is supported by UKRI-BBSRC (grants BB/ R009031/1 and BB/T003804/1), a BBSRC-EPSRC Impact Accelerator grant and a Wellcome Trust early concept development grant (University of Glasgow).
Status:Published
Refereed:Yes
Glasgow Author(s) Enlighten ID:Tokatlidis, Professor Kostas and Lacko, Mr Erik
Authors: Dimogkioka, A.-R., Lees, J., Tokatlidis, K., and Lacko, E.
College/School:College of Medical Veterinary and Life Sciences > Institute of Molecular Cell and Systems Biology
Journal Name:RSC Advances
Publisher:Royal Society of Chemistry
ISSN:2046-2069
Published Online:01 October 2021
Copyright Holders:Copyright © 2021 The Royal Society of Chemistry
First Published:First published in RSC Advances 11(51): 32476-32493
Publisher Policy:Reproduced under a Creative Commons License

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Project CodeAward NoProject NamePrincipal InvestigatorFunder's NameFunder RefLead Dept
300402Mitochondria salvage via a novel antioxidant protein import pathwayKonstantinos TokatlidisBiotechnology and Biological Sciences Research Council (BBSRC)BB/R009031/1Institute of Molecular, Cell & Systems Biology
306567Mitochondrial ROS mapping and control with sub-organellar resolutionKonstantinos TokatlidisBiotechnology and Biological Sciences Research Council (BBSRC)BB/T003804/1Institute of Molecular, Cell & Systems Biology