1.2.1.25: branched-chain alpha-keto acid dehydrogenase system
This is an abbreviated version!
For detailed information about branched-chain alpha-keto acid dehydrogenase system, go to the full flat file.
Word Map on EC 1.2.1.25
-
1.2.1.25
-
maple
-
syrup
-
pdha1
-
dihydrolipoamide
-
alpha2beta2
-
transacylase
-
keto-acids
-
bckdhb
-
dihydrolipoyl
- 1.2.1.25
-
maple
- syrup
- pdha1
- dihydrolipoamide
-
alpha2beta2
-
transacylase
-
keto-acids
- bckdhb
-
dihydrolipoyl
Reaction
Synonyms
2-oxoisovalerate dehydrogenase, alpha-ketoisovalerate dehydrogenase, BCKD, BCKD complex, BCKDalpha, BCKDbeta, BCKDC, BCKDH, BCKDHA, BCKDHB, bkdA, BKDH, branched chain alpha-keto acid dehydrogenase complex, branched chain keto-acid dehydrogenase, branched-chain alpha-keto acid dehydrogenase complex, branched-chain alpha-ketoacid dehydrogenase, branched-chain alpha-ketoacid dehydrogenase complex, branched-chain alpha-ketoacid dehydrogenase complex E1alpha subunit, branched-chain ketoacid dehydrogenase, branched-chain-keto-acid dehydrogenase alpha, branched-chain-keto-acid dehydrogenase beta, dehydrogenase, 2-oxoisovalerate (acylating), E1alpha subunit, E1beta subunit, mitochondrial branched-chain keto-acid dehydrogenase, More
ECTree
1.2.1.25 branched-chain alpha-keto acid dehydrogenase systemAdvanced search results
results (
results (Natural Substrates Products
Natural Substrates Products on EC 1.2.1.25 - branched-chain alpha-keto acid dehydrogenase system
Please wait a moment until all data is loaded. This message will disappear when all data is loaded.
top
NATURAL SUBSTRATE 
NATURAL PRODUCT
REACTION DIAGRAM
ORGANISM
UNIPROT
COMMENTARY
(Substrate)
(Substrate)
LITERATURE
(Substrate)
(Substrate)
COMMENTARY
(Product)
(Product)
LITERATURE
(Product)
(Product)
REVERSIBILITY
r=reversible
ir=irreversible
?=not specified
r=reversible
ir=irreversible
?=not specified
3-methyl-2-oxobutanoate + CoA + NAD+
2-methylpropanoyl-CoA + CO2 + NADH
-
-
-
ir
3-methyl-2-oxopentanoate + CoA + NAD+
2-methylbutanoyl-CoA + CO2 + NADH
-
-
-
ir
4-methyl-2-oxopentanoate + CoA + NAD+
3-methylbutanoyl-CoA + CO2 + NADH
-
-
-
ir
additional information
?
-
the human BCKD complex catalyzes the irreversible oxidative decarboxylation of the branched-chain ketoacids (2-keto-isocaproate derived from leucine, 2-keto-3-methylglutarate obtained from isoleucine, and 2-keto-isovalerate from valine). This reaction produces CO2, NADH, and the respective branched-chain acyl-coA intermediates with a 1:1:1 stoichiometry. The action of BCKD complex generates isovaleryl-CoA from 2-ketoisocaproate (leucine), 2-methylbutyryl-CoA from 2-keto-3-methylglutarate (isoleucine), and isobutyryl-CoA from 2-keto-isovalerate (valine)
-
-
-
3-methyl-2-oxobutanoate + CoA + NAD+
2-methylpropanoyl-CoA + CO2 + NADH + H+
-
-
-
-
ir
3-methyl-2-oxobutanoate + CoA + NAD+
2-methylpropanoyl-CoA + CO2 + NADH + H+
-
-
-
ir
3-methyl-2-oxobutanoate + CoA + NAD+
2-methylpropanoyl-CoA + CO2 + NADH + H+
-
-
-
-
ir
3-methyl-2-oxobutanoate + CoA + NAD+
2-methylpropanoyl-CoA + CO2 + NADH + H+
-
-
-
ir
3-methyl-2-oxobutanoate + CoA + NAD+
2-methylpropanoyl-CoA + CO2 + NADH + H+
-
-
-
-
ir
3-methyl-2-oxobutanoate + CoA + NAD+
2-methylpropanoyl-CoA + CO2 + NADH + H+
-
-
-
ir
3-methyl-2-oxobutanoate + CoA + NAD+
2-methylpropanoyl-CoA + CO2 + NADH + H+
-
-
-
-
ir
3-methyl-2-oxobutanoate + CoA + NAD+
2-methylpropanoyl-CoA + CO2 + NADH + H+
-
-
-
-
ir
3-methyl-2-oxobutanoate + CoA + NAD+
2-methylpropanoyl-CoA + CO2 + NADH + H+
-
-
-
?
3-methyl-2-oxopentanoate + CoA + NAD+
2-methylbutanoyl-CoA + CO2 + NADH + H+
-
-
-
-
ir
3-methyl-2-oxopentanoate + CoA + NAD+
2-methylbutanoyl-CoA + CO2 + NADH + H+
-
-
-
ir
3-methyl-2-oxopentanoate + CoA + NAD+
2-methylbutanoyl-CoA + CO2 + NADH + H+
-
-
-
-
ir
3-methyl-2-oxopentanoate + CoA + NAD+
2-methylbutanoyl-CoA + CO2 + NADH + H+
-
-
-
ir
3-methyl-2-oxopentanoate + CoA + NAD+
2-methylbutanoyl-CoA + CO2 + NADH + H+
-
-
-
-
ir
3-methyl-2-oxopentanoate + CoA + NAD+
2-methylbutanoyl-CoA + CO2 + NADH + H+
-
-
-
ir
3-methyl-2-oxopentanoate + CoA + NAD+
2-methylbutanoyl-CoA + CO2 + NADH + H+
-
-
-
-
ir
3-methyl-2-oxopentanoate + CoA + NAD+
2-methylbutanoyl-CoA + CO2 + NADH + H+
-
-
-
-
ir
4-methyl-2-oxopentanoate + CoA + NAD+
3-methylbutanoyl-CoA + CO2 + NADH + H+
-
-
-
-
ir
4-methyl-2-oxopentanoate + CoA + NAD+
3-methylbutanoyl-CoA + CO2 + NADH + H+
-
-
-
ir
4-methyl-2-oxopentanoate + CoA + NAD+
3-methylbutanoyl-CoA + CO2 + NADH + H+
-
-
-
-
ir
4-methyl-2-oxopentanoate + CoA + NAD+
3-methylbutanoyl-CoA + CO2 + NADH + H+
-
-
-
ir
4-methyl-2-oxopentanoate + CoA + NAD+
3-methylbutanoyl-CoA + CO2 + NADH + H+
-
-
-
-
ir
4-methyl-2-oxopentanoate + CoA + NAD+
3-methylbutanoyl-CoA + CO2 + NADH + H+
-
-
-
ir
4-methyl-2-oxopentanoate + CoA + NAD+
3-methylbutanoyl-CoA + CO2 + NADH + H+
-
-
-
-
ir
4-methyl-2-oxopentanoate + CoA + NAD+
3-methylbutanoyl-CoA + CO2 + NADH + H+
-
-
-
-
ir
