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.1) 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)
Immunogen
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.
Western blot analysis in NCI-H292 cell line lysates (35ug/lane).
Application Notes
For WB starting dilution is: 1:1000
Additional Information
Form
Liquid
Storage Instructions
Store at 4˚C for three months and -20˚C, stable for up to one year. As with all antibodies care should be taken to avoid repeated freeze thaw cycles. Antibodies should not be exposed to prolonged high temperatures.