Mitochondrial pyruvate carboxylase
Cofactors used
Clinical / pharmacological. PC deficiency: lactic acidosis, hypoglycaemia, neurological disease
What it does, reaction by reaction
Gluconeogenesis Carbohydrate Metabolism · Cytosol + mitochondrion + ER
Pyruvate + HCO3− + ATP → oxaloacetate + ADP + Pi
Converts Pyruvate HCO3- ATP into Oxaloacetate ADP Pi
› Notes
Mitochondrial pyruvate carboxylase requires covalently bound biotin, ATP, Mg2+, and bicarbonate as the CO2 source. This is the first bypass of pyruvate kinase and is effectively irreversible; it is allosterically activated by acetyl-CoA, linking fatty-acid oxidation to glucose production.
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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