|Application ||WB, IHC-P, E|
|Other Accession||NP_001091972.1, NP_653237.1|
|Calculated MW||15996 Da|
|Antigen Region||101-129 aa|
|Other Names||Mitochondrial intermembrane space import and assembly protein 40, Coiled-coil-helix-coiled-coil-helix domain-containing protein 4, CHCHD4, MIA40|
|Target/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.|
|Format||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.|
|Precautions||MIA40 Antibody (C-term) is for research use only and not for use in diagnostic or therapeutic procedures.|
|Function||Functions as chaperone and catalyzes the formation of disulfide bonds in substrate proteins, such as COX17 or MICU1 (PubMed:16185709, PubMed:26387864, PubMed:19182799, PubMed:21059946, PubMed:23186364). Required for the import and folding of small cysteine-containing proteins (small Tim) in the mitochondrial intermembrane space (IMS) (PubMed:16185709, PubMed:19182799, PubMed:21059946). Precursor proteins to be imported into the IMS are translocated in their reduced form into the mitochondria (PubMed:16185709, PubMed:19182799, PubMed:21059946). 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). 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).|
|Cellular Location||Mitochondrion intermembrane space|
|Tissue Location||Expressed in all tissues tested, suggesting an ubiquitous expression.|
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Provided below are standard protocols that you may find useful for product applications.
CHCHD4, a component of human mitochondria, belongs to a protein family whose members share 6 highly conserved cysteine residues constituting a -CXC-CX(9)C-CX(9)C- motif in the C terminus (Hofmann et al., 2005 [PubMed 16185709]).
Daithankar, V.N., et al. Biochemistry 48(22):4828-4837(2009)
Chacinska, A., et al. J. Biol. Chem. 283(44):29723-29729(2008)
Terziyska, N., et al. FEBS Lett. 581(6):1098-1102(2007)
Hofmann, S., et al. J. Mol. Biol. 353(3):517-528(2005)
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