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

Nazanine Modjtahedi; Kostas Tokatlidis; Philippe Dessen; Guido Kroemer

doi:10.1016/j.tibs.2015.12.004

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

Nazanine Modjtahedi, Kostas Tokatlidis, Philippe Dessen, Guido Kroemer

Résultats de recherche: Contribution à un journal › Article 'review' › Revue par des pairs

92 Citations (Scopus)

Résumé

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.

langue originale	Anglais
Pages (de - à)	245-260
Nombre de pages	16
journal	Trends in Biochemical Sciences
Volume	41
Numéro de publication	3
Les DOIs	https://doi.org/10.1016/j.tibs.2015.12.004
état	Publié - 1 mars 2016

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10.1016/j.tibs.2015.12.004

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title = "Mitochondrial Proteins Containing Coiled-Coil-Helix-Coiled-Coil-Helix (CHCH) Domains in Health and Disease",

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.",

keywords = "Cancer, Disulfide relay, Metabolic disorder, Mitochondrion, Protein import, Respiratory chain",

author = "Nazanine Modjtahedi and Kostas Tokatlidis and Philippe Dessen and Guido Kroemer",

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AU - Modjtahedi, Nazanine

AU - Tokatlidis, Kostas

AU - Dessen, Philippe

AU - Kroemer, Guido

PY - 2016/3/1

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N2 - 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.

AB - 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.

KW - Cancer

KW - Disulfide relay

KW - Metabolic disorder

KW - Mitochondrion

KW - Protein import

KW - Respiratory chain

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