Methylcrotonoyl-CoA Carboxylase Subunit Alpha, Mitochondrial

**Semantic type:** Amino Acid, Peptide, or Protein|Enzyme

**Definition:** Methylcrotonoyl-CoA carboxylase subunit alpha, mitochondrial (725 aa, ~80 kDa) is encoded by the human MCCC1 gene. This protein plays a role in the conversion of 3-methylcrotonyl-CoA to 3-methylglutaconyl-CoA.

**Synonyms:** - 3-Methylcrotonyl-CoA Carboxylase 1 - 3-Methylcrotonyl-CoA Carboxylase Biotin Containing Subunit - 3-Methylcrotonyl-CoA Carboxylase Biotin-Containing Subunit - 3-Methylcrotonyl-CoA:Carbon Dioxide Ligase Subunit Alpha - EC 6.4.1.4 - MCCC1 - MCCase Subunit Alpha - Methylcrotonoyl-CoA Carboxylase 1