| Literature DB >> 26782138 |
Nazanine Modjtahedi1, Kostas Tokatlidis2, Philippe Dessen3, Guido Kroemer4.
Abstract
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.Entities:
Keywords: cancer; disulfide relay; metabolic disorder; mitochondrion; protein import; respiratory chain
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Year: 2016 PMID： 26782138 DOI： 10.1016/j.tibs.2015.12.004
Source DB: PubMed Journal: Trends Biochem Sci ISSN： 0968-0004 Impact factor: 13.807