Gene:
methylcrotonoyl-Coenzyme A carboxylase 2 (beta)
Gene ID:
197592
Symbol:
MCCC2
Species:
1000
Description:
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.