Functions as chaperone and catalyzes the formation of disulfide bonds in substrate proteins, such as COX17, COX19 and MICU1 (PubMed:16185709, PubMed:26387864, PubMed:19182799, PubMed:21059946, PubMed:23186364, PubMed:23676665).
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 (PubMed:16185709, PubMed:19182799, PubMed:21059946, PubMed:23676665).
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).
Applications:Suitable for use in Western Blot, Immunohistochemistry, Immunofluorescence/Immunocytochemistry and ELISA.
Other applications not tested.
Recommended Dilution:Western Blot: 1:500-1:2000, incubate membrane with diluted antibody in TBS, 5% milk, 0.1% Tween20 at 4°C with gentle shaking, overnight
Immunohistochemistry: 1:50-1:200Immunofluorescence (IF-IC): 1:100-1:500ELISA (peptide): 1:20,000-1:40,000Optimal dilutions to be determined by the researcher.
Storage and Stability:May be stored at 4°C for short-term only.
Aliquot to avoid repeated freezing and thawing.
Store at -20°C.
Aliquots are stable for 12 months after receipt.
For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.
仕様
Size：100ul
Host：rabbit
Source Antibody：human
Grade：Affinity Purified
Purity：Purified by immunoaffinity chromatography from serum.
Form：Supplied as a liquid in PBS, pH 7.4, 0.02% sodium azide, 50% glycerol.
Specificity：Recognizes endogenous levels of human CHCHD4. Species Crossreactivity: mouse and rat
Isotype：IgG
Calc Applications Abbrev：E IC IF IHC WB
Calc Crossreactivity：Hu Mo Rt
Immunogen：Synthetic peptide corresponding to human CHCHD4. Species sequence homology: Porcine (92%), Zebrafish (83%), Bovine (92%), Equine (92%), Sheep (92%), Rabbit (92%), Canine (92%), Chicken (92%), Xenopus (92%)
