Branched-chain alpha-ketoacid dehydrogenase complex (BCKDH)
Cofactors used
Clinical / pharmacological. Maple syrup urine disease: burnt-sugar urine, ketoacidosis; thiamine-responsive variants
What it does, reaction by reaction
Amino Acid Catabolism Overview (glucogenic vs. ketogenic amino acids; brief overview of branched-chain amino acid catabolism and phenylalanine/tyrosine catabolism as representative examples) Amino Acid & Nitrogen Metabolism · Cytosol + mitochondrion
Branched-chain α-keto acid + CoA + NAD⁺ → branched-chain acyl-CoA + CO₂ + NADH
Converts CoA-SH NAD+ into CO2 NADH
› Notes
The mitochondrial branched-chain α-ketoacid dehydrogenase complex (BCKDH) catalyzes irreversible oxidative decarboxylation. It requires TPP (vitamin B1), lipoamide, FAD (vitamin B2), NAD⁺ (vitamin B3), CoA (vitamin B5), and Mg²⁺; this is the committed, rate-limiting step of BCAA oxidation and produces one NADH per keto acid.
Showing all 1 reactions.
What accelerates and inhibits it
Regulation is pathway-specific, so each context is listed separately
No regulation recorded for this enzyme in the source documents.
Recent literature
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