|Other Names||Methylcrotonoyl-CoA carboxylase subunit alpha, mitochondrial, MCCase subunit alpha, 3-methylcrotonyl-CoA carboxylase 1, 3-methylcrotonyl-CoA carboxylase biotin-containing subunit, 3-methylcrotonyl-CoA:carbon dioxide ligase subunit alpha, MCCC1, MCCA|
|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||Biotin-attachment 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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Provided below are standard protocols that you may find useful for product applications.
MCCC1 is the large subunit of3-methylcrotonyl-CoA carboxylase. This enzyme functions as aheterodimer and catalyzes the carboxylation of 3-methylcrotonyl-CoAto form 3-methylglutaconyl-CoA.
Holzinger, A., et al. Hum. Mol. Genet. 10(12):1299-1306(2001)Obata, K., et al. Genomics 72(2):145-152(2001)Baumgartner, M.R., et al. J. Clin. Invest. 107(4):495-504(2001)Gallardo, M.E., et al. Am. J. Hum. Genet. 68(2):334-346(2001)
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