COMPARTMENTS

PCCB [ENSP00000419027]

Propionyl-CoA carboxylase beta chain, mitochondrial; 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 (By similarity). 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 (By similarity).

Synonyms:  PCCB,  C9JAW3,  C9JQS9,  C9JVW9,  C9JVY9 ...

Linkouts:  STRING  Pharos  UniProt  OMIM