Loss of Propionyl-CoA Carboxylase Reprograms Hepatic Metabolism by Suppressing Mitochondrial Pyruvate Carboxylation and Fatty Acid Oxidation
This study demonstrates that Propionyl-CoA Carboxylase deficiency in hepatocytes reprograms hepatic metabolism by suppressing mitochondrial pyruvate carboxylation and fatty acid oxidation while increasing glucose oxidation, thereby impairing gluconeogenesis and lipid synthesis and highlighting the metabolic risks of prolonged fasting in Propionic Acidemia patients.