TPP cofactor: branched-chain alpha-ketoacid dehydrogenase
- Required for leucine, isoleucine and valine catabolism
- The enzyme deficient in maple syrup urine disease
- BCKA DH clears the ketoacids of leucine, isoleucine, valine
- Its congenital failure is maple syrup urine disease; thiamine-responsive variants exist
Drugs with this mechanism (1)
- Thiamine pyrophosphate — Active thiamine coenzyme required for carbohydrate metabolism; deficiency impairs energy production and contributes to Wernicke encephalopathy.
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