| Function | This is one of the 2 subunits of the biotin-dependent propionyl-CoA carboxylase (PCC), a mitochondrial enzyme involved in the catabolism of odd chain fatty acids, branched-chain amino acids isoleucine, threonine, methionine, and valine and other metabolites. Propionyl-CoA carboxylase catalyzes the carboxylation of propionyl-CoA/propanoyl-CoA to D-methylmalonyl-CoA/(S)-methylmalonyl-CoA. Within the holoenzyme, the alpha subunit catalyzes the ATP-dependent carboxylation of the biotin carried by the biotin carboxyl carrier (BCC) domain, while the beta subunit then transfers the carboxyl group from carboxylated biotin to propionyl-CoA. Propionyl-CoA carboxylase also significantly acts on butyryl-CoA/butanoyl-CoA, which is converted to ethylmalonyl-CoA/(2S)-ethylmalonyl-CoA at a much lower rate. Other alternative minor substrates include (2E)-butenoyl-CoA/crotonoyl-CoA. |
| Protein Name | Propionyl-Coa Carboxylase Alpha Chain - MitochondrialPccase Subunit AlphaPropanoyl-Coa -Carbon Dioxide Ligase Subunit Alpha |
| Database Links | Reactome: R-HSA-196780Reactome: R-HSA-3371599Reactome: R-HSA-71032 |
| Cellular Localisation | Mitochondrion Matrix |
| Alternative Protein Names | Propionyl-Coa Carboxylase Alpha Chain - Mitochondrial proteinPccase Subunit Alpha proteinPropanoyl-Coa -Carbon Dioxide Ligase Subunit Alpha proteinPCCA protein |
Information sourced from Uniprot.org
