Propionyl-CoA carboxylase (PCC)
Cofactors used
Clinical / pharmacological. Propionic acidaemia; biotin-dependent
What it does, reaction by reaction
Propionyl-CoA to Succinyl-CoA (anaplerosis) Amino Acid & Nitrogen Metabolism · Mitochondrial matrix
Propionyl-CoA + HCO3- + ATP -> D-methylmalonyl-CoA + ADP + Pi
Converts Propionyl-CoA HCO3- ATP into D-Methylmalonyl-CoA ADP Pi
› Notes
Entry point for odd-chain fatty acids, valine, isoleucine, methionine and threonine. Deficiency causes propionic acidaemia.
Showing all 1 reactions.
What accelerates and inhibits it
Regulation is pathway-specific, so each context is listed separately
No regulation recorded for this enzyme in the source documents.
Recent literature
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