Chchd4
entrez
72170
canonical
uniprot
Q8VEA4
canonical
ensembl
ENSMUSG00000034203
resolver
ensp
ENSMUSP00000041380
resolver
enst
ENSMUST00000040835
resolver
genesymbol
Chchd4
resolver
genesymbol_syn
Mia40
resolver
uniprot_entry
MIA40_MOUSE
resolver
amino_acid_sequence
MSYCRQEGKDRIIFVTKEDHETPSSAELVADDPNDPYEEHGLILPNGDINWNCPCLGGMASGPCGEQFKSAFSCFHYSTEDIKGSDCIDQFRAMQECMQKYPDLYPQDEEEEEEAKPVEPVEETADTKVSAAKEQGTSS
uniprot
proteins
function
FUNCTION: Central component of a redox-sensitive mitochondrial intermembrane space import machinery which is required for the biogenesis of respiratory chain complexes (PubMed:26004228). Functions as a chaperone and catalyzes the formation of disulfide bonds in substrate proteins, such as COA7, COX17, COX19, MICU1 and TIMCC/FAM136A. Required for the import and folding of small cysteine-containing proteins (small Tim) in the mitochondrial intermembrane space (IMS). Required for the import of COA7 in the 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. Mediates formation of disulfide bond in MICU1 in the IMS, promoting formation of the MICU1-MICU2 heterodimer that regulates mitochondrial calcium uptake. {ECO:0000250|UniProtKB:Q8N4Q1, ECO:0000269|PubMed:26004228}.
uniprot
proteins
mass_dalton
15525
uniprot
proteins
post_translational_modification
PTM: Forms intrachain disulfide bridges, but exists in different redox states. {ECO:0000250}.
uniprot
proteins
pubmed
15489334
uniprot
proteins
pubmed
16141072
uniprot
proteins
pubmed
16185709
uniprot
proteins
pubmed
21183079
uniprot
proteins
pubmed
26004228
uniprot
proteins
sequence_length
139
uniprot
proteins
subcellular_location
SUBCELLULAR LOCATION: Mitochondrion intermembrane space {ECO:0000250|UniProtKB:Q8N4Q1}.
uniprot
proteins