MIA40 Antibody (C-term) [APR08447G]

MIA40 Antibody (C-term) [APR08447G] | Gentaur UK, US & Europe Distribution

Product Category: Polyclonal Antibodies

Host: Rabbit

Species Reactivity: H

Specificity: This MIA40 antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide between 101-129 amino acids from the C-terminal region of human MIA40.

Cellular Localisation: Mitochondrion intermembrane space

Molecular Weight: 15996

Clone: Polyclonal

Gene Name: CHCHD4

Gene ID: 131474

Function: Central component of a redox-sensitive mitochondrial intermembrane space import machinery which is required for the biogenesis of respiratory chain complexes (PubMed:26004228) . Functions as chaperone and catalyzes the formation of disulfide bonds in substrate proteins, such as COX17, COX19, MICU1 and COA7 (PubMed:16185709, PubMed:26387864, PubMed:19182799, PubMed:21059946, PubMed:23186364, PubMed:23676665, PubMed: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 (PubMed: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 (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) .

Summary: Tissue Location: Expressed in all tissues tested, suggesting an ubiquitous expression.

Form: Purified polyclonal antibody supplied in PBS with 0.09% (W/V) sodium azide. This antibody is purified through a protein A column, followed by peptide affinity purification.

Storage: Maintain refrigerated at 2-8°C for up to 2 weeks. For long term storage store at -20°C in small aliquots to prevent freeze-thaw cycles.

Application: WB, IHC-P

Dilution: WB--1:1000 IHC-P--1:100~500

Synonyms: Mitochondrial intermembrane space import and assembly protein 40, Coiled-coil-helix-coiled-coil-helix domain-containing protein 4, CHCHD4, MIA40