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Schematic diagram of oxidative decarboxylation

For pyruvate dehydrogenase (R=H). For alpha-ketoglurate (R=CH2-COO-). Step A is the E1k-mediated decarboxylation step, utilizing TPP as a cofactor. Steps B and C are E2k-mediated sequential succinylation steps, ultimately forming succinyl CoA and utilizing lipoamide and coenzyme A as cofactors. Steps D and E are E3-mediated electron transfer reactions that utilize FAD and NAD as cofactors. (Courtesy of Wikimedia Commons. Public domain.)

Associated Disorders

  • DOOR syndrome
  • Dihydrolipoyl dehydrogenase deficiency
  • Eronen syndrome
  • Ketoacidotic crisis
  • Lactic acidosis
  • Maple syrup urine disease