|Application ||WB, E|
|Calculated MW||80473 Da|
|Antigen Region||397-425 aa|
|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|
|Target/Specificity||This MCCC1 antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide between 397-425 amino acids from the Central region of human MCCC1.|
|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||MCCC1 Antibody (Center) is for research use only and 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 of 3-methylcrotonyl-CoA carboxylase. This enzyme functions as a heterodimer and catalyzes the carboxylation of 3-methylcrotonyl-CoA to 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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