|Other Names||Methylcrotonoyl-CoA carboxylase beta chain, mitochondrial, MCCase subunit beta, 3-methylcrotonyl-CoA carboxylase 2, 3-methylcrotonyl-CoA carboxylase non-biotin-containing subunit, 3-methylcrotonyl-CoA:carbon dioxide ligase subunit beta, MCCC2, MCCB|
|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||Carboxyltransferase subunit of the 3-methylcrotonyl-CoA carboxylase, an enzyme that catalyzes the conversion of 3- methylcrotonyl-CoA to 3-methylglutaconyl-CoA, a critical step for leucine and isovaleric acid catabolism.|
|Cellular Location||Mitochondrion matrix|
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This gene encodes the small subunit of3-methylcrotonyl-CoA carboxylase. This enzyme functions as aheterodimer and catalyzes the carboxylation of 3-methylcrotonyl-CoAto form 3-methylglutaconyl-CoA. Mutations in this gene areassociated with 3-Methylcrotonylglycinuria, an autosomal recessivedisorder of leucine catabolism.
Yoshida, T., et al. Int. J. Mol. Med. 25(4):649-656(2010)Oguri, M., et al. Am. J. Hypertens. 23(1):70-77(2010)Cho, Y.S., et al. Proteins 70(1):268-272(2008)Uematsu, M., et al. J. Hum. Genet. 52(12):1040-1043(2007)Stadler, S.C., et al. Biochem. Biophys. Res. Commun. 334(3):939-946(2005)
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