Description
Product Usage Information
Western Blotting: 1:1000
Simple Western™: 1:50 - 1:250
Immunoprecipitation: 1:50
Storage
Supplied in 10 mM sodium HEPES (pH 7.5), 150 mM NaCl, 100 µg/mL BSA, 50% glycerol, and less than 0.02% sodium azide. Store at -20°C. Do not aliquot the antibody.
Protocol
Available protocols: Western Blotting, Immunoprecipitation
Specificity / Sensitivity
MIA40/CHCHD4 (F8J4L) Rabbit Monoclonal Antibody recognizes endogenous levels of total MIA40/CHCHD4 protein.
Species Reactivity: Human
Source / Purification
Monoclonal antibody is produced by immunizing animals with a synthetic peptide corresponding to residues surrounding Ala128 of human MIA40/CHCHD4 protein.
Background
Mitochondrial intermembrane space import and assembly protein 40 (MIA40), also known as coiled-coil-helix-coiled-coil-helix domain-containing protein 4 (CHCHD4), is a chaperone and oxidoreductase in the mitochondrial intermembrane space (IMS). It is an import receptor for various intermembrane space proteins and has a key role in their oxidative folding (1,2). Studies show that the IMS protein AIFM1 mediates the import of MIA40/CHCHD4. Furthermore, AIFM1 forms a stable complex with MIA40/CHCHD4. This complex is crucial for the import of MIA40/CHCHD4 substrates into the IMS (1). In addition, the mitochondrial inner-membrane-associated protein C9orf72 is imported into the IMS via AIFM1-MIA40/CHCHD4 pathway. C9orf72 then stabilizes the assembly of mitochondrial complex I (3).
Alternate Names
CHCHD4; coiled-coil-helix-coiled-coil-helix domain containing 4; Coiled-coil-helix-coiled-coil-helix domain-containing protein 4; FLJ31709; MIA40; Mitochondrial intermembrane space import and assembly protein 40; TIMM40; translocase of inner mitochondrial membrane 40 homolog
