Anti-CHCHD4 Antibody

Catalog No : IGX-41662
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Description Central component of a redox-sensitive mitochondrial intermembrane space import machinery which is required for the biogenesis of respiratory chain complexes (PMID:26004228). Functions as chaperone and catalyzes the formation of disulfide bonds in substrate proteins, such as COX17, COX19, MICU1 and COA7 (PMID:16185709, PMID:26387864, PMID:19182799, PMID:21059946, PMID:23186364, PMID:23676665, PMID:30885959). 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 (PMID:30885959). 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 (PMID:16185709, PMID:19182799, PMID:21059946, PMID:23676665). Reduced CHCHD4/MIA40 is then reoxidized by GFER/ERV1 via a disulfide relay system (PMID:23186364). Mediates formation of disulfide bond in MICU1 in the IMS, promoting formation of the MICU1-MICU2 heterodimer that regulates mitochondrial calcium uptake (PMID:26387864).
Other names Mitochondrial intermembrane space import and assembly protein 40, Coiled-coil-helix-coiled-coil-helix domain-containing protein 4, CHCHD4, MIA40
Cellular localization Mitochondrion Intermembrane Space
Research area Metabolism
Note This product is for research purpose only, not for use in diagnostics or as therapeutics.
Uniprot ID Q8N4Q1
Product name Anti-CHCHD4 Antibody
Catalog No IGX-41662
Supplier’s Catalog No IGX-41662
Supplier ImuGeX
Host species Rabbit
Species reactivity Human
Applications ELISA, IHC
Recommended dilutions IHC: 1:20-1:200
Molecular weight N/A
Conjugate Unconjugated
Immunogen Recombinant Human Mitochondrial intermembrane space import and assembly protein 40 protein (1-142AA)
Specificity CHCHD4 antibody detects endogenous level of CHCHD4 protein
Storage Store at +4°C for short term (1 week), for long term aliquote and store at -20°C or -80°C. Avoid repeated freeze and thaw cycles, with each freeze and thaw cycle an antibody can lose half of its binding activity.
Clonality Polyclonal
Isotype IgG
Clone ID N/A
Concentration 1 mg/mL
Storage buffer Phosphate-buffered saline (pH 7.4) containing 0.03% Proclin and 50% Glycerol.
Formulation Liquid
Purification This antibody is immunogen affinity purified.
Handling This product contains Proclin as preservative, please take appropriate care when handling this product.
Reactivity life 12 months when stored properly according to instructions (provided antibody remains sterile).