Edwards, R., Eaglesfield, R. and Tokatlidis, K. (2021) The mitochondrial intermembrane space: the most constricted mitochondrial sub-compartment with the largest variety of protein import pathways. Open Biology, 11(3), 210002. (doi: 10.1098/rsob.210002) (PMID:33715390) (PMCID:PMC8061763)
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Abstract
The mitochondrial intermembrane space (IMS) is the most constricted sub-mitochondrial compartment, housing only about 5% of the mitochondrial proteome, and yet is endowed with the largest variability of protein import mechanisms. In this review, we summarize our current knowledge of the major IMS import pathway based on the oxidative protein folding pathway and discuss the stunning variability of other IMS protein import pathways. As IMS-localized proteins only have to cross the outer mitochondrial membrane, they do not require energy sources like ATP hydrolysis in the mitochondrial matrix or the inner membrane electrochemical potential which are critical for import into the matrix or insertion into the inner membrane. We also explore several atypical IMS import pathways that are still not very well understood and are guided by poorly defined or completely unknown targeting peptides. Importantly, many of the IMS proteins are linked to several human diseases, and it is therefore crucial to understand how they reach their normal site of function in the IMS. In the final part of this review, we discuss current understanding of how such IMS protein underpin a large spectrum of human disorders.
| Item Type: | Articles |
|---|---|
| Status: | Published |
| Refereed: | Yes |
| Glasgow Author(s) Enlighten ID: | Tokatlidis, Professor Kostas and Edwards, Mr Ruairidh and Eaglesfield, Mr Ross |
| Authors: | Edwards, R., Eaglesfield, R., and Tokatlidis, K. |
| College/School: | College of Medical Veterinary and Life Sciences > School of Molecular Biosciences College of Medical Veterinary and Life Sciences > School of Life Sciences |
| Journal Name: | Open Biology |
| Publisher: | The Royal Society |
| ISSN: | 2046-2441 |
| ISSN (Online): | 2046-2441 |
| Published Online: | 10 March 2021 |
| Copyright Holders: | Copyright © 2021 The Authors |
| First Published: | First published in Open Biology 11(3): 210002 |
| Publisher Policy: | Reproduced under a Creative Commons License |
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