TPP cofactor: branched-chain alpha-ketoacid dehydrogenase
- Required for leucine, isoleucine and valine catabolism
- BCKA DH clears the ketoacids of leucine, isoleucine, valine
- The enzyme deficient in 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.
Self-check
- Which three amino acids depend on this catabolic pathway?
Leucine, isoleucine and valine. - Are any maple-syrup-urine-disease variants thiamine-responsive?
Yes.
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