|Other Names||Mitochondrial intermembrane space import and assembly protein 40, Coiled-coil-helix-coiled-coil-helix domain-containing protein 4, CHCHD4, MIA40|
|Format||Synthetic peptide was lyophilized with 100% acetonitrile and is supplied as a powder. Reconstitute with 0.1 ml DI water for a final concentration of 1 mg/ml.|
|Storage||Maintain refrigerated at 2-8°C for up to 6 months. For long term storage store at -20°C.|
|Precautions||This product is for research use only. 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, 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).|
|Cellular Location||Mitochondrion intermembrane space Mitochondrion|
|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 aprotein family whose members share 6 highly conserved cysteineresidues 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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