InnateDB: Systems Biology of the Innate Immune Response

Homo sapiens Protein: CHCHD4

Summary

InnateDB Protein

IDBP-236511.6

Last Modified

2014-10-13 [Report errors or provide feedback]

Gene Symbol

CHCHD4

Protein Name

coiled-coil-helix-coiled-coil-helix domain containing 4

Synonyms

Species

Homo sapiens

Ensembl Protein

ENSP00000380122

InnateDB Gene

IDBG-19928 (CHCHD4)

Protein Structure

UniProt Annotation

Function

Functions as chaperone and catalyzes the formation of disulfide bonds in substrate proteins, such as COX17. Required for the import and folding of small cysteine-containing proteins (small Tim) in the mitochondrial intermembrane space (IMS). Precursor proteins to be imported into the IMS are translocated in their reduced form into the mitochondria. The oxidized form of CHCHD4/MIA40 forms a transient intermolecular disulfide bridge with the reduced precursor protein, resulting in oxidation of the precursor protein that now contains an intramolecular disulfide bond and is able to undergo folding in the IMS. Reduced CHCHD4/MIA40 is then reoxidized by GFER/ERV1 via a disulfide relay system. {ECO:0000269PubMed:16185709, ECO:0000269PubMed:19182799, ECO:0000269PubMed:21059946, ECO:0000269PubMed:23186364}.

Subcellular Localization

Mitochondrion intermembrane space {ECO:0000269PubMed:16185709, ECO:0000269PubMed:23186364}.

Disease Associations

Tissue Specificity

Expressed in all tissues tested, suggesting an ubiquitous expression. {ECO:0000269PubMed:16185709}.

Comments

Interactions

Number of Interactions

This gene and/or its encoded proteins are associated with 18 experimentally validated interaction(s) in this database.

Experimentally validated
Total	18 [view]
Protein-Protein	18 [view]
Protein-DNA	0
Protein-RNA	0
DNA-DNA	0
RNA-RNA	0
DNA-RNA	0

Gene Ontology

Molecular Function

Accession	GO Term
GO:0015035	protein disulfide oxidoreductase activity

Biological Process

GO:0006626	protein targeting to mitochondrion
GO:0022417	protein maturation by protein folding
GO:0044267	cellular protein metabolic process
GO:0045041	protein import into mitochondrial intermembrane space
GO:0051084	'de novo' posttranslational protein folding
GO:0055114	oxidation-reduction process