Propionyl-CoA Carboxylase Alpha Chain, Mitochondrial

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

**Definition:** Propionyl-CoA carboxylase alpha chain, mitochondrial (728 aa, ~80 kDa) is encoded by the human PCCA gene. This protein is involved in the carboxylation of biotin to promote the carboxylation of butynyl-CoA and propionyl-CoA.

**Synonyms:** - EC 6.4.1.3 - PCCA - PCCase Alpha Subunit - PCCase Subunit Alpha - Propanoyl-CoA:Carbon Dioxide Ligase Alpha Subunit - Propanoyl-CoA:Carbon Dioxide Ligase Subunit Alpha - Propionyl-CoA Carboxylase Alpha Chain