Mitochondrial Proteins Containing Coiled-Coil-Helix-Coiled-Coil-Helix (CHCH) Domains in Health and Disease

Nazanine Modjtahedi, Kostas Tokatlidis, Philippe Dessen, Guido Kroemer

    Research output: Contribution to journalReview articlepeer-review

    93 Citations (Scopus)

    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.

    Original languageEnglish
    Pages (from-to)245-260
    Number of pages16
    JournalTrends in Biochemical Sciences
    Volume41
    Issue number3
    DOIs
    Publication statusPublished - 1 Mar 2016

    Keywords

    • Cancer
    • Disulfide relay
    • Metabolic disorder
    • Mitochondrion
    • Protein import
    • Respiratory chain

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