Mitochondrial proteins containing coiled-coil-helix-coiled-coil-helix (CHCH) domains in health and disease

Modjtahedi, N., Tokatlidis, K. , Dessen, P. and Kroemer, G. (2016) Mitochondrial proteins containing coiled-coil-helix-coiled-coil-helix (CHCH) domains in health and disease. Trends in Biochemical Sciences, 41(3), pp. 245-260. (doi:10.1016/j.tibs.2015.12.004) (PMID:26782138)

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Members of the coiled-coil-helix-coiled-coil-helix (CHCH) domain-containing protein family that carry (CX9C) type motifs are imported into the mitochondrion with the help of the disulfide relay-dependent MIA import pathway. These evolutionarily conserved proteins are emerging as new cellular factors that control mitochondrial respiration, redox regulation, lipid homeostasis, and membrane ultrastructure and dynamics. We discuss recent insights on the activity of known (CX9C) motif-carrying proteins in mammals and review current data implicating the Mia40/CHCHD4 import machinery in the regulation of their mitochondrial import. Recent findings and the identification of disease-associated mutations in specific (CX9C) motif-carrying proteins have highlighted members of this family of proteins as potential therapeutic targets in a variety of human disorders.

Item Type:Articles
Glasgow Author(s) Enlighten ID:Tokatlidis, Professor Kostas
Authors: Modjtahedi, N., Tokatlidis, K., Dessen, P., and Kroemer, G.
College/School:College of Medical Veterinary and Life Sciences > Institute of Molecular Cell and Systems Biology
Journal Name:Trends in Biochemical Sciences
Publisher:Elsevier (Cell Press)
ISSN (Online):0167-7640
Published Online:15 January 2016
Copyright Holders:Copyright © 2016 Elsevier (Cell Press)
First Published:First published in Trends in Biochemical Sciences 41(3):245-260
Publisher Policy:Reproduced in accordance with the copyright policy of the publisher

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