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Proteins/UNIPROT:P21953
Together with BCKDHA forms the heterotetrameric E1 subunit of the mitochondrial branched-chain alpha-ketoacid dehydrogenase (BCKD) complex. The BCKD complex catalyzes the multi-step oxidative decarboxylation of alpha-ketoacids derived from the branched-chain amino-acids valine, leucine and isoleucine producing CO2 and acyl-CoA which is subsequently utilized to produce energy. The E1 subunit catalyzes the first step with the decarboxylation of the alpha-ketoacid forming an enzyme-product intermediate. A reductive acylation mediated by the lipoylamide cofactor of E2 extracts the acyl group from the E1 active site for the next step of the reaction..
Function from UniProtKB (P21953), read 2026-09-23. CC BY 4.0.
Curated compounds routed: 0
6 rows
34 statements
34 statements · 6 reference databases · Subject: BCKDHB · Sources: Hetionet, Human-GEM, Reactome, Rhea, UniProt, WikiPathways
11 reactions
2-oxobutanoate+proton+thiamine(1+) diphosphatecarbon dioxide+2-(alpha-Hydroxypropyl)thiamine diphosphate
Reaction pageLeft to rightBalance undeterminableEC 1.2.4.4mitochondrial matrix
Human-GEM MAR03800CC BY 4.0Reference data
MAM02393m+2-methyl-1-hydroxypropylthiamine diphosphateMAM02857m+thiamine(1+) diphosphate
Catalysed by DBT
3 names
Resolves to: BCKDHB
Reaction pageLeft to rightBalance undeterminableEC 1.2.4.4mitochondrial matrix
Human-GEM MAR06416CC BY 4.0Reference data
MAM02393m+MAM20079mMAM02856m+thiamine(1+) diphosphate
Catalysed by DBT & BCKDHA & BCKDHB
Reaction pageLeft to rightBalance undeterminableEC 1.2.4.4mitochondrial matrix
Human-GEM MAR06419CC BY 4.0Reference data
MAM02393m+3-methyl-1-hydroxybutylthiamine diphosphateMAM02858m+thiamine(1+) diphosphate
Catalysed by DBT & BCKDHA & BCKDHB
Reaction pageLeft to rightBalance undeterminableEC 1.2.4.4mitochondrial matrix
Human-GEM MAR06421CC BY 4.0Reference data
3-methyl-2-oxobutanoate+proton+thiamine(1+) diphosphatecarbon dioxide+2-methyl-1-hydroxypropylthiamine diphosphate
Reaction pageLeft to rightBalance undeterminableEC 1.2.4.4mitochondrial matrix
Human-GEM MAR20174CC BY 4.0Reference data
3-methyl-2-oxovaleric acid+proton+thiamine(1+) diphosphatecarbon dioxide+MAM20079m
Reaction pageLeft to rightBalance undeterminableEC 1.2.4.4mitochondrial matrix
Human-GEM MAR20175CC BY 4.0Reference data
4-methyl-2-oxopentanoate+proton+thiamine(1+) diphosphatecarbon dioxide+3-methyl-1-hydroxybutylthiamine diphosphate
Reaction pageLeft to rightBalance undeterminableEC 1.2.4.4mitochondrial matrix
Human-GEM MAR20176CC BY 4.0Reference data
MAM02393m+2-(alpha-Hydroxypropyl)thiamine diphosphatethiamine(1+) diphosphate+MAM20082m
Catalysed by DBT & BCKDHA & BCKDHB
Reaction pageLeft to rightBalance undeterminableEC 1.2.4.4mitochondrial matrix
Human-GEM MAR20177CC BY 4.0Reference data
N(6)-[(R)-lipoyl]-L-lysyl-[protein]+3-methyl-2-oxobutanoate+hydronN(6)-[(R)-S(8)-2-methylpropanoyldihydrolipoyl]-L-lysyl-[protein]+carbon dioxide
Reaction pageLeft to rightBalance undeterminable
Rhea RHEA:13457CC BY 4.0Reference data
N(6)-[(R)-lipoyl]-L-lysyl-[protein]+4-methyl-2-oxopentanoate+hydronN(6)-[(R)-S(8)-3-methylbutanoyldihydrolipoyl]-L-lysyl-[protein]+carbon dioxide
Reaction pageLeft to rightBalance undeterminable
Rhea RHEA:84639CC BY 4.0Reference data
N(6)-[(R)-lipoyl]-L-lysyl-[protein]+(S)-3-methyl-2-oxopentanoate+hydronN(6)-{(R)-S(8)-[(2S)-2-methylbutanoyl]dihydrolipoyl}-L-lysyl-[protein]+carbon dioxide
Reaction pageLeft to rightBalance undeterminable
Rhea RHEA:84643CC BY 4.0Reference data