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Proteins/UNIPROT:P11182
The branched-chain alpha-keto dehydrogenase complex catalyzes the overall conversion of alpha-keto acids to acyl-CoA and CO(2). It contains multiple copies of three enzymatic components: branched-chain alpha-keto acid decarboxylase (E1), lipoamide acyltransferase (E2) and lipoamide dehydrogenase (E3). Within this complex, the catalytic function of this enzyme is to accept, and to transfer to coenzyme A, acyl groups that are generated by the branched-chain alpha-keto acid decarboxylase component..
Function from UniProtKB (P11182), read 2026-09-23. CC BY 4.0.
Curated compounds routed: 0
7 rows
39 statements
39 statements · 5 reference databases · Subject: DBT · Sources: Hetionet, Human-GEM, Reactome, UniProt, WikiPathways
11 reactions
MAM02393m+2-methyl-1-hydroxypropylthiamine diphosphateMAM02857m+thiamine(1+) diphosphate
Catalysed by DBT & BCKDHA & BCKDHB
Reaction pageLeft to rightBalance undeterminableEC 1.2.4.4mitochondrial matrix
Human-GEM MAR06416CC BY 4.0Reference data
CHEBI:1146900+MAM02857mMAM01701m+isobutyryl-CoA
Catalysed by DBT
Reaction pageReversibleBalancedEC 2.3.1.168mitochondrial matrix
12 names
Resolves to: DBT
Human-GEM MAR06417CC 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
CHEBI:1146900+MAM02856mCHEBI:15477+MAM01701m
Catalysed by DBT
Reaction pageLeft to rightBalancedEC 2.3.1.168mitochondrial matrix
Human-GEM MAR06420CC 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
CHEBI:1146900+MAM02858mMAM01701m+isovaleryl-CoA
Catalysed by DBT
Reaction pageLeft to rightBalancedEC 2.3.1.168mitochondrial matrix
Human-GEM MAR06422CC 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
CHEBI:1146900+MAM20082mMAM01701m+propionyl-CoA
Catalysed by DBT
Reaction pageLeft to rightUnbalancedEC 1.2.4.4mitochondrial matrix
Human-GEM MAR20178CC BY 4.0Reference data
N(6)-[(R)-dihydrolipoyl]-L-lysyl-[protein]+2-methylpropanoyl-CoAN(6)-[(R)-S(8)-2-methylpropanoyldihydrolipoyl]-L-lysyl-[protein]+coenzyme A(4-)
Catalysed by DBT
Reaction pageRight to leftBalance undeterminable
Rhea RHEA:18865CC BY 4.0Reference data
N(6)-[(R)-dihydrolipoyl]-L-lysyl-[protein]+isovaleryl-CoAN(6)-[(R)-S(8)-3-methylbutanoyldihydrolipoyl]-L-lysyl-[protein]+coenzyme A(4-)
Catalysed by DBT
Reaction pageRight to leftBalance undeterminable
Rhea RHEA:84647CC BY 4.0Reference data
CHEBI:88166+N(6)-[(R)-dihydrolipoyl]-L-lysyl-[protein]N(6)-{(R)-S(8)-[(2S)-2-methylbutanoyl]dihydrolipoyl}-L-lysyl-[protein]+coenzyme A(4-)
Catalysed by DBT
Reaction pageRight to leftBalance undeterminable
Rhea RHEA:84651CC BY 4.0Reference data