UniProt Accession

 MIA40_RAT; Q5BJN5 

Genbank Protein ID

 BC091407 

Genbank Nucleotide ID

 AAH91407.1 

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
116	EEEEEEAKPVEPVEE	acetylation	[1]

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

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. 

Interpro

 IPR010625; CHCH. 

Pfam

 PF06747; CHCH 

SMART

  

PROSITE

  

PRINTS