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Thiamin pyrophosphate (TPP) and its component parts

Thiamin pyrophosphate (TPP) and its component parts: the pyrimidine and thiazole rings, and the pyrophosphate (or “diphosphate”) moiety. The diphosphate moiety should not be confused with two phosphates. TPP has a dissociable proton on the thiazole ring; when this proton is extracted it leaves behind an especially reactive and negatively charged carbon atom (a carbanion) in that ring. (Courtesy of Wikimedia Commons. Modified by D J Lanska.)

Associated Disorders

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