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InnateDB Protein
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IDBP-170129.6
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Last Modified
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2014-10-13 [Report errors or provide feedback]
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Gene Symbol
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Chchd4
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Protein Name
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coiled-coil-helix-coiled-coil-helix domain containing 4
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Synonyms
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2410012P20Rik; 2810014D17Rik; AI838740;
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Species
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Mus musculus
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Ensembl Protein
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ENSMUSP00000041380
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InnateDB Gene
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IDBG-170127 (Chchd4)
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Protein Structure
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| 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}.
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| Subcellular Localization |
Mitochondrion intermembrane space {ECO:0000250}.
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| Disease Associations |
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| Tissue Specificity |
Widely expressed. Present at high level in liver and kidney, followed by lung, brain, heart and spleen (at protein level). {ECO:0000269PubMed:16185709}.
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| Comments |
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Number of Interactions
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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.
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| Predicted by orthology |
| Total |
15 [view]
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Molecular Function |
| Accession |
GO Term |
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GO:0015035
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protein disulfide oxidoreductase activity
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| Biological Process |
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GO:0022417
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protein maturation by protein folding
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GO:0045041
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protein import into mitochondrial intermembrane space
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GO:0051084
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'de novo' posttranslational protein folding
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GO:0055114
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oxidation-reduction process
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| Cellular Component |
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| PDB ID |
MGI:1919420
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| InterPro |
IPR010625
CHCH
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| PFAM |
PF06747
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| PRINTS |
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| PIRSF |
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| SMART |
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| TIGRFAMs |
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| Modification |
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| SwissProt |
Q8VEA4
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| PhosphoSite |
PhosphoSite-
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| TrEMBL |
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| UniProt Splice Variant |
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| Entrez Gene |
72170
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| UniGene |
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| RefSeq |
NP_598689
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| MGI ID |
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| MGI Symbol |
Chchd4
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| OMIM |
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| CCDS |
CCDS20367
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| HPRD |
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| IMGT |
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| EMBL |
AK012745
BC019405
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| GenPept |
AAH19405
BAC25375
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Version 5.4