BackgroundFunctions 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.
DescriptionCHCHD4 Polyclonal Antibody. Unconjugated. Raised in: Rabbit.
FormulationLiquid. 0.03% Proclin 300, 50% Glycerol, 0.01M PBS, PH 7.4.
SpecificityHuman
IsotypeIgG
Uniprot IDQ8N4Q1
Purification>95%, Protein G purified
ImmunogenRecombinant Human Mitochondrial intermembrane space import and assembly protein 40 protein (1-142AA)
StorageShipped at 4°C. Upon delivery aliquot and store at -20°C (short-term) or -80°C (long-term). Avoid repeated freeze.
Alternative NamesCoiled-coil-helix-coiled-coil-helix domain-containing protein 4, CHCHD4, MIA40
ApplicationELISA, IHC; Recommended dilution: IHC:1:20-1:200