Official Full Name
methylcrotonoyl-CoA carboxylase 1 (alpha)
This gene encodes 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. Mutations in this gene are associated with 3-Methylcrotonylglycinuria, an autosomal recessive disorder of leucine catabolism.
MCCC1; methylcrotonoyl-CoA carboxylase 1 (alpha); methylcrotonoyl Coenzyme A carboxylase 1 (alpha); methylcrotonoyl-CoA carboxylase subunit alpha, mitochondrial; MCCA; 3-methylcrotonyl-CoA carboxylase 1; MCCase subunit alpha; OTTHUMP00000212827; OTTHUMP00000212828; OTTHUMP00000212829; OTTHUMP00000212832; methylcrotonoyl-Coenzyme A carboxylase 1 (alpha); 3-methylcroto; MCC-B; 3-methylcrotonyl-CoA:carbon dioxide ligase subunit alpha; 3-methylcrotonyl-CoA carboxylase biotin-containing subunit; FLJ25545; DKFZp686B20267; zgc:154106; si:dkey-37m8.6