catalog number :
MBS9133187
products full name :
CHCHD4 Polyclonal Antibody
products short name :
[CHCHD4]
products name syn :
[MIA40; TIMM40]
other names :
[mitochondrial intermembrane space import and assembly protein 40 isoform 1; Mitochondrial intermembrane space import and assembly protein 40; mitochondrial intermembrane space import and assembly protein 40; coiled-coil-helix-coiled-coil-helix domain containing 4; Coiled-coil-helix-coiled-coil-helix domain-containing protein 4]
products gene name :
[CHCHD4]
other gene names :
[CHCHD4; CHCHD4; MIA40; TIMM40; MIA40]
reactivity :
Human, Mouse
sequence :
MSYCRQEGKDRIIFVTKEDHETPSSAELVADDPNDPYEE
HGLILPNGNINWNCPCLGGMASGPCGEQFKSAFSCFHYS
TEEIKGSDCVDQFRAMQECMQKYPDLYPQEDEDEEEERE
KKPAEQAEETAPIEATATKEEEGSS
purity :
Affinity Purification
storage stability :
Store at -20°C. Avoid freeze / thaw cycles.
tested application :
Western Blot (WB)
app notes :
WB: 1:500 - 1:2000
image1 heading :
Western Blot (WB)
other info1 :
Immunogen: Recombinant fusion protein containing a sequence corresponding to amino acids 1-142 of human CHCHD4 (NP_001091972.1)
other info2 :
Buffer: PBS with 0.02% sodium azide, 50% glycerol, pH7.3. Calculated MW: 15kDa/17kDa. Observed MW: 20kDa
products categories :
Polyclonal Antibodies
ncbi acc num :
NP_001091972.1
ncbi gb acc num :
NM_001098502.1
ncbi pathways :
Metabolism Of Proteins Pathway (106230); Mitochondrial Protein Import Pathway (576261)
ncbi summary :
CHCHD4, a component of human mitochondria, belongs to a protein family whose members share 6 highly conserved cysteine residues constituting a -CXC-CX(9)C-CX(9)C- motif in the C terminus (Hofmann et al., 2005 [PubMed 16185709]).[supplied by OMIM, Mar 2008]
uniprot summary :
Functions as chaperone and catalyzes the formation of disulfide bonds in substrate proteins, such as COX17 or MICU1 (PubMed:16185709, PubMed:26387864, PubMed:19182799, PubMed:21059946, PubMed:23186364). Required for the import and folding of small cysteine-containing proteins (small Tim) in the mitochondrial intermembrane space (IMS) (PubMed:16185709, PubMed:19182799, PubMed:21059946). Precursor proteins to be imported into the IMS are translocated in their reduced form into the mitochondria (PubMed:16185709, PubMed:19182799, PubMed:21059946). 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 (PubMed:16185709, PubMed:19182799, PubMed:21059946). Reduced CHCHD4/MIA40 is then reoxidized by GFER/ERV1 via a disulfide relay system (PubMed:23186364). Mediates formation of disulfide bond in MICU1 in the IMS, promoting formation of the MICU1-MICU2 heterodimer that regulates mitochondrial calcium uptake (PubMed:26387864).