Tag | Content |
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CPLM ID | CPLM-013536 |
UniProt Accession | |
Genbank Protein ID | |
Genbank Nucleotide ID | |
Protein Name | Mitochondrial intermembrane space import and assembly protein 40 |
Protein Synonyms/Alias | Coiled-coil-helix-coiled-coil-helix domain-containing protein 4 |
Gene Name | Chchd4 |
Gene Synonyms/Alias | Mia40 |
Created Date | July 27, 2013 |
Organism | Rattus norvegicus (Rat) |
NCBI Taxa ID | 10116 |
Lysine Modification | Position | Peptide | Type | References |
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116 | EEEEEEAKPVEPVEE | acetylation | [1] |
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Reference | [1] Proteomic analysis of lysine acetylation sites in rat tissues reveals organ specificity and subcellular patterns. Lundby A, Lage K, Weinert BT, Bekker-Jensen DB, Secher A, Skovgaard T, Kelstrup CD, Dmytriyev A, Choudhary C, Lundby C, Olsen JV. Cell Rep. 2012 Aug 30;2(2):419-31. [ PMID: 22902405] |
Functional Description | 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). |
Sequence Annotation | DOMAIN 57 100 CHCH. DISULFID 53 55 Redox-active (By similarity). DISULFID 64 97 By similarity. DISULFID 74 87 By similarity. |
Keyword | Complete proteome; Disulfide bond; Mitochondrion; Oxidoreductase; Protein transport; Redox-active center; Reference proteome; Translocation; Transport. |
Sequence Source | UniProt (SWISSPROT/TrEMBL); GenBank; EMBL |
Protein Length | 139 AA |
Protein Sequence | MSYCRQEGKD RIIFVTKEDH ETPSSAELVA DDPNDPYEEH GLILPNGDIN WNCPCLGGMA 60 SGPCGEQFKS AFSCFHYSTE DIKGSDCIDQ FRAMQECMQK YPDLYPQDEE EEEEAKPVEP 120 VEETADTKAS AAKEQGASS 139 |
Gene Ontology | GO:0005758; C:mitochondrial intermembrane space; ISS:UniProtKB. GO:0015035; F:protein disulfide oxidoreductase activity; ISS:UniProtKB. GO:0051084; P:'de novo' posttranslational protein folding; ISS:UniProtKB. GO:0045041; P:protein import into mitochondrial intermembrane space; ISS:UniProtKB. GO:0022417; P:protein maturation by protein folding; ISS:UniProtKB. |
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