|Application ||WB, E|
|Calculated MW||61333 Da|
|Antigen Region||468-496 aa|
|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|
|Target/Specificity||This MCCC2 antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide between 468-496 amino acids from the C-terminal region of human MCCC2.|
|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||MCCC2 Antibody (C-term) is for research use only and 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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Provided below are standard protocols that you may find useful for product applications.
This gene encodes the small 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. Mutations in this gene are associated with 3-Methylcrotonylglycinuria, an autosomal recessive disorder 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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