Propionyl-CoA carboxylase
Clinical / pharmacological. Propionic acidaemia; biotin-dependent
What it does, reaction by reaction
Pyrimidine Degradation Nucleotide Metabolism · Cytosol
Propionyl-CoA → succinyl-CoA
Converts Propionyl-CoA into Succinyl-CoA
› Notes
Propionyl-CoA carboxylase uses ATP, bicarbonate, and biotin to convert propionyl-CoA to D-methylmalonyl-CoA; methylmalonyl-CoA epimerase produces L-methylmalonyl-CoA; and adenosylcobalamin-dependent methylmalonyl-CoA mutase rearranges it to succinyl-CoA. These downstream steps permit thymine carbon to enter the tricarboxylic acid cycle.
Showing all 1 reactions.
What accelerates and inhibits it
Regulation is pathway-specific, so each context is listed separately
Amino Acid Catabolism Overview (glucogenic vs. ketogenic amino acids; brief overview of branched-chain amino acid catabolism and phenylalanine/tyrosine catabolism as representative examples)
Propionyl-CoA availability; biotin sufficiency
Biotin deficiency; product accumulation
No dominant acute hormonal switch; flux follows odd-chain fatty-acid and amino-acid catabolism
Pyrimidine Degradation
Listed there as: Propionyl-CoA carboxylase (downstream thymine-carbon handling)
Propionyl-CoA and bicarbonate availability
Biotin deficiency and loss-of-function variants impair activity
Metabolic/nutritional state influences substrate flow; no defining acute hormonal allostery.
Recent literature
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