Chchd4
entrez
312559
canonical
uniprot
Q5BJN5
canonical
ensembl
ENSRNOG00000022466
resolver
ensembl
ENSRNOG00000069157
resolver
ensembl
ENSRNOG00055004336
resolver
ensembl
ENSRNOG00055032119
resolver
ensembl
ENSRNOG00060027251
resolver
ensembl
ENSRNOG00060030779
resolver
ensembl
ENSRNOG00065024628
resolver
ensembl
ENSRNOG00065031009
resolver
ensp
ENSRNOP00000038359
resolver
ensp
ENSRNOP00000094363
resolver
ensp
ENSRNOP00055005186
resolver
ensp
ENSRNOP00055045799
resolver
ensp
ENSRNOP00060039383
resolver
ensp
ENSRNOP00060044413
resolver
ensp
ENSRNOP00065034581
resolver
ensp
ENSRNOP00065043932
resolver
enst
ENSRNOT00000038096
resolver
enst
ENSRNOT00000116242
resolver
enst
ENSRNOT00055006889
resolver
enst
ENSRNOT00055055511
resolver
enst
ENSRNOT00060047313
resolver
enst
ENSRNOT00060053459
resolver
enst
ENSRNOT00065042280
resolver
enst
ENSRNOT00065053444
resolver
genesymbol
Chchd4
resolver
genesymbol_syn
Mia40
resolver
uniprot_entry
MIA40_RAT
resolver
amino_acid_sequence
MSYCRQEGKDRIIFVTKEDHETPSSAELVADDPNDPYEEHGLILPNGDINWNCPCLGGMASGPCGEQFKSAFSCFHYSTEDIKGSDCIDQFRAMQECMQKYPDLYPQDEEEEEEAKPVEPVEETADTKASAAKEQGASS
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. 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}.
uniprot
proteins
mass_dalton
15467
uniprot
proteins
post_translational_modification
PTM: Forms intrachain disulfide bridges, but exists in different redox states. {ECO:0000250}.
uniprot
proteins
pubmed
15489334
uniprot
proteins
sequence_length
139
uniprot
proteins
subcellular_location
SUBCELLULAR LOCATION: Mitochondrion intermembrane space {ECO:0000250|UniProtKB:Q8N4Q1}.
uniprot
proteins