Description
In some thiamine-responsive variants of MSUD, the branched-chain alpha-keto acid dehydrogenase complex possesses a structural defect that lowers its affinity for its essential cofactor, thiamine pyrophosphate. Flooding the system with high doses of natural thiamine forces the cofactor into the defective enzyme, significantly restoring its ability to break down toxic branched-chain amino acids and preventing sudden neurological crises.
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