Anti-MCCC2, CT (MCCC2, MCCB, Methylcrotonoyl-CoA carboxylase beta chain, mitochondrial, 3-methylcrot

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. Applications: Suitable for use in Western Blot, ELISA, Recommended Dilution: ELISA: 1:1,000, Western Blot: 1:100-500, Storage and Stability: May be stored at 4°C for short-term only. Aliquot to avoid repeated freezing and thawing. Store at -20°C. Aliquots are stable for 12 months. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.