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The reaction of TPP-E1k with alpha-ketoglutarate

Note: R1 includes the pyrimidine ring, and R2 includes the pyrophosphate moiety. (Courtesy of Wikimedia Commons. Creative Commons Attribution-Share Alike 4.0 International license.)

Associated Disorders

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