Version 5.4
Bos taurus Protein: CHCHD4
Summary
InnateDB Protein IDBP-699256.2
Last Modified 2014-10-13 [Report errors or provide feedback]
Gene Symbol CHCHD4
Protein Name Mitochondrial intermembrane space import and assembly protein 40
Synonyms
Species Bos taurus
Ensembl Protein ENSBTAP00000020635
InnateDB Gene IDBG-646450 (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 (By similarity). {ECO:0000250}.
Subcellular Localization Mitochondrion intermembrane space {ECO:0000250}.
Disease Associations
Tissue Specificity
Comments
Interactions
Number of Interactions This gene and/or its encoded proteins are associated with 0 experimentally validated interaction(s) in this database.
They are also associated with 15 interaction(s) predicted by orthology.
Predicted by orthology
Total 15 [view]
Gene Ontology

Molecular Function
Accession GO Term
GO:0015035 protein disulfide oxidoreductase activity
GO:0016491 oxidoreductase activity
Biological Process
GO:0006810 transport
GO:0015031 protein transport
GO:0022417 protein maturation by protein folding
GO:0045041 protein import into mitochondrial intermembrane space
GO:0051084 'de novo' posttranslational protein folding
GO:0055114 oxidation-reduction process
Cellular Component
GO:0005739 mitochondrion
GO:0005758 mitochondrial intermembrane space
Protein Structure and Domains
PDB ID
InterPro IPR009069 Cysteine alpha-hairpin motif superfamily
IPR010625 CHCH
PFAM PF06747
PRINTS
PIRSF
SMART
TIGRFAMs
Post-translational Modifications
Modification
Cross-References
SwissProt Q2KHZ4
PhosphoSite PhosphoSite-
TrEMBL
UniProt Splice Variant
Entrez Gene 513889
UniGene Bt.11014
RefSeq NP_001039624
HUGO
OMIM
CCDS
HPRD
IMGT
EMBL BC112827
GenPept AAI12828

If you use InnateDB for your research, please cite the following publication:

Breuer et al., InnateDB: systems biology of innate immunity and beyond - recent updates and continuing curation. Nucl. Acids Res. (2013) 41 (D1) 

InnateDB is being developed jointly by the Brinkman Laboratory (Simon Fraser University, British Columbia, Canada), the Hancock Laboratory (University of British Columbia, Vancouver, British Columbia) and the Lynn EMBL Australia Group (South Australian Health & Medical Research Institute and Flinders University, Adelaide, Australia).

Funding is currently provided by Allergen and EMBL Australia. Previous funding has been provided by Genome Canada, the Foundation for the National Institutes of Health through the Grand Challenges in Global Health initiative and by Teagasc. InnateDB curated interactions are licensed under the Design Science License. All other data is licensed under the terms of the originating database. Contact: innatedb-mail@sfu.ca