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
Europe PMC · fetched just now · sorted by publication date
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1
Mitochondrial NADPH fuels mitochondrial fatty acid synthesis and lipoylation to power oxidative metabolism.
Kim D, Kesavan R, Ryu K, Dey T, Marckx A, Menezes C, Praharaj PP, Morley S, Ko B, Soflaee MH, Tom HJ, Brown H… · 2025-04-21
cited 20× unreviewed -
2
Apparent Saturation of Branched-Chain Amino Acid Catabolism After High Dietary Milk Protein Intake in Healthy Adults.
Newton-Tanzer E, Can SN, Demmelmair H, Horak J, Holdt L, Koletzko B, Grote V. · 2025-05-01
cited 2× open access unreviewed -
3
The role of BCAA metabolism in metabolic health and disease.
Choi BH, Hyun S, Koo SH. · 2024-07-02
cited 91× open access unreviewed -
4
ATF3/SPI1/SLC31A1 Signaling Promotes Cuproptosis Induced by Advanced Glycosylation End Products in Diabetic Myocardial Injury.
Huo S, Wang Q, Shi W, Peng L, Jiang Y, Zhu M, Guo J, Peng D, Wang M, Men L, Huang B, Lv J, Lin L. · 2023-01-14
cited 185× open access unreviewed -
5
Gut microbiota and fermentation-derived branched chain hydroxy acids mediate health benefits of yogurt consumption in obese mice.
Daniel N, Nachbar RT, Tran TTT, Ouellette A, Varin TV, Cotillard A, Quinquis L, Gagné A, St-Pierre P, Trottie… · 2022-03-15
cited 58× open access unreviewed -
6
Tumour-reprogrammed stromal BCAT1 fuels branched-chain ketoacid dependency in stromal-rich PDAC tumours.
Zhu Z, Achreja A, Meurs N, Animasahun O, Owen S, Mittal A, Parikh P, Lo TW, Franco-Barraza J, Shi J, Gunchick… · 2020-07-06
cited 187× open access unreviewed -
7
Genetics and genomic medicine in Iran.
Behnam B, Zakeri M. · 2019-02-01
cited 8× open access unreviewed -
8
Protein lipoylation: an evolutionarily conserved metabolic regulator of health and disease.
Rowland EA, Snowden CK, Cristea IM. · 2017-11-21
cited 180× unreviewed -
9
Mitochondrial health, the epigenome and healthspan.
Aon MA, Cortassa S, Juhaszova M, Sollott SJ. · 2016-08-01
cited 57× unreviewed -
10
A diagnostic approach for cerebral palsy in the genomic era.
Lee RW, Poretti A, Cohen JS, Levey E, Gwynn H, Johnston MV, Hoon AH, Fatemi A. · 2014-10-04
cited 64× unreviewed
External claims. These come from an index outside this database and are not checked against it. Treat them as leads.