Methylcrotonoyl-CoA carboxylase subunit alpha, mitochondrial (MCCC1)

Biotin-attachment subunit of the 3-methylcrotonyl-CoA carboxylase, an enzyme which catalyzes the conversion of 3- methylcrotonyl-CoA into 3-methylglutaconyl-CoA, a critical step for leucine and isovaleric acid catabolism. .