1.2.4.4: 3-methyl-2-oxobutanoate dehydrogenase (2-methylpropanoyl-transferring) This is an abbreviated version! For detailed information about 3-methyl-2-oxobutanoate dehydrogenase (2-methylpropanoyl-transferring), go to the full flat file .
Reaction
3-methyl-2-oxobutanoate +
[dihydrolipoyllysine-residue (2-methylpropanoyl)transferase] lipoyllysine =
[dihydrolipoyllysine-residue (2-methylpropanoyl)transferase] S-(2-methylpropanoyl)dihydrolipoyllysine +
CO2 +
2 H+
Synonyms 2-oxoisocaproate dehydrogenase, 2-oxoisovalerate dehydrogenase, 2-oxoisovalerate dehydrogenase (lipoate), 2-oxoisovalerate dehydrogenase subunit alpha, alpha-keto-beta-methylvalerate dehydrogenase, alpha-ketoacid dehydrogenase, alpha-ketoisocaproate dehydrogenase, alpha-ketoisocaproic dehydrogenase, alpha-ketoisocaproic-alpha-keto-beta-methylvaleric dehydrogenase, alpha-ketoisovalerate dehydrogenase, alpha-oxoisocaproate dehydrogenase, BCDH, BCKA dehydrogenase, BCKADC, BCKD, BCKDC, BCKDH, BCKDH E1-alpha, BCKDH E1-beta, BCKDHA, BCKDHB, BCKDHc, BCOA, BCOAD, BDKG, Bkd, bkdF, branched chain alpha-ketoacid dehydrogenase complex, branched chain keto acid dehydrogenase, branched-chain (-2-oxoacid BCD) dehydrogenase, branched-chain 2-keto acid dehydrogenase, branched-chain 2-oxo acid dehydrogenase, branched-chain alpha-keto acid decarboxylase/dehydrogenase, branched-chain alpha-keto acid dehydrogenase, branched-chain alpha-keto acid dehydrogenase complex, branched-chain alpha-keto acid dehydrogenase E1beta subunit, branched-chain alpha-ketoacid dehydrogenase, branched-chain alpha-ketoacid dehydrogenase complex, branched-chain alpha-ketoacid dehydrogenase enzyme complex, branched-chain alpha-ketoacid dehydrogenase multienzyme complex, branched-chain alpha-oxo acid dehydrogenase, branched-chain keto acid dehydrogenase, branched-chain keto acid dehydrogenase complex, branched-chain ketoacid dehydrogenase, branched-chain ketoacid dehydrogenase E1, dehydrogenase, 2-oxoisocaproate, dehydrogenase, 2-oxoisovalerate (lipoate), dehydrogenase, branched chain alpha-keto acid, DHalpha, dihydrolipoyl acyl-transferase, dihydrolipoyl transacylase, E1b, E1b component, E1b component of the 2-oxo acid dehydrogenase complex, E2, EC 1.2.4.3, More
ECTree
Cofactor
Cofactor on EC 1.2.4.4 - 3-methyl-2-oxobutanoate dehydrogenase (2-methylpropanoyl-transferring)
Please wait a moment until all data is loaded. This message will disappear when all data is loaded.
Please wait a moment until the data is sorted. This message will disappear when the data is sorted.
CoA
-
-
CoA
-
-
349052 , 349053 , 349060 , 349067 , 349068 , 349070 , 349074 , 349077 , 349079 , 349085 , 349088 , 349090 , 587061 , 710845
CoA
-
-
94884 , 349050 , 349051 , 349055 , 349057 , 349058 , 349059 , 349063 , 349064 , 349075 , 710845
CoA
-
the multienzyme complex catalyzes a thiamine diphosphate- and Mg2+-dependent oxidative decarboxylation of branched-chain keto acids with the formation of branched-chain acyl-CoA and reduction of NAD+ to NADH: R1R2CHCOCOOH + NAD+ + CoASH = R1R2CHCO-S-CoA + NADH + CO2, component E1 (EC 1.2.4.4, branched-chain keto acid dehydrogenase), component E2 (no EC number, branched-chain acyltransferase), component E3 (EC 1.8.1.4, dihydrolipoamide dehydrogenase), partial reactions catalyzed by the multienzyme complex: 1. R1R2CHCOCOOH + E1-(thiamine diphosphate) = E1-(thiamine diphosphate)-COHCHR1R2 + CO2, 2. E1-(thiamine diphosphate)-COHCHR1R2 + lipoyl-E2 = R1R2CHCO-SCoA + dihydrolipoyl-E2, 4. dihydrolipoyl-E2 + NAD+ = lipoyl-E2 + NADH
CoA
-
the multienzyme complex catalyzes a thiamine diphosphate- and Mg2+-dependent oxidative decarboxylation of branched-chain keto acids with the formation of branched-chain acyl-CoA and reduction of NAD+ to NADH: R1R2CHCOCOOH + NAD+ + CoASH = R1R2CHCO-S-CoA + NADH + CO2, component E1 (EC 1.2.4.4, branched-chain keto acid dehydrogenase), component E2 (no EC number, branched-chain acyltransferase), component E3 (EC 1.8.1.4, dihydrolipoamide dehydrogenase), partial reactions catalyzed by the multienzyme complex: 1. R1R2CHCOCOOH + E1-(thiamine diphosphate) = E1-(thiamine diphosphate)-COHCHR1R2 + CO2, 2. E1-(thiamine diphosphate)-COHCHR1R2 + lipoyl-E2 = R1R2CHCO-SCoA + dihydrolipoyl-E2, 4. dihydrolipoyl-E2 + NAD+ = lipoyl-E2 + NADH
CoA
-
the multienzyme complex catalyzes a thiamine diphosphate- and Mg2+-dependent oxidative decarboxylation of branched-chain keto acids with the formation of branched-chain acyl-CoA and reduction of NAD+ to NADH: R1R2CHCOCOOH + NAD+ + CoASH = R1R2CHCO-S-CoA + NADH + CO2, component E1 (EC 1.2.4.4, branched-chain keto acid dehydrogenase), component E2 (no EC number, branched-chain acyltransferase), component E3 (EC 1.8.1.4, dihydrolipoamide dehydrogenase), partial reactions catalyzed by the multienzyme complex: 1. R1R2CHCOCOOH + E1-(thiamine diphosphate) = E1-(thiamine diphosphate)-COHCHR1R2 + CO2, 2. E1-(thiamine diphosphate)-COHCHR1R2 + lipoyl-E2 = R1R2CHCO-SCoA + dihydrolipoyl-E2, 4. dihydrolipoyl-E2 + NAD+ = lipoyl-E2 + NADH
CoA
-
extraordinarily tightly bound
CoA
-
Km: 0.0071 mM in the reaction with 4-methyl-2-oxopentanoate, Km: 0.009 mM in the reaction with 3-methyl-2-oxobutanoate, Km: 0.007 in the reaction with DL-3-methyl-2-oxopentanoate
NAD+
-
-
NAD+
-
-
349052 , 349053 , 349060 , 349067 , 349068 , 349070 , 349074 , 349077 , 349079 , 349085 , 349088 , 349090 , 587061 , 710845 , 723788 , 741079
NAD+
-
-
94884 , 349050 , 349051 , 349055 , 349057 , 349058 , 349059 , 349061 , 349063 , 349064 , 349075 , 710845
NAD+
-
the multienzyme complex catalyzes a thiamine diphosphate- and Mg2+-dependent oxidative decarboxylation of branched-chain keto acids with the formation of branched-chain acyl-CoA and reduction of NAD+ to NADH: R1R2CHCOCOOH + NAD+ + CoASH = R1R2CHCO-S-CoA + NADH + CO2, component E1 (EC 1.2.4.4, branched-chain keto acid dehydrogenase), component E2 (no EC number, branched-chain acyltransferase), component E3 (EC 1.8.1.4, dihydrolipoamide dehydrogenase), partial reactions catalyzed by the multienzyme complex: 1. R1R2CHCOCOOH + E1-(thiamine diphosphate) = E1-(thiamine diphosphate)-COHCHR1R2 + CO2, 2. E1-(thiamine diphosphate)-COHCHR1R2 + lipoyl-E2 = R1R2CHCO-SCoA + dihydrolipoyl-E2, 4. dihydrolipoyl-E2 + NAD+ = lipoyl-E2 + NADH
NAD+
-
the multienzyme complex catalyzes a thiamine diphosphate- and Mg2+-dependent oxidative decarboxylation of branched-chain keto acids with the formation of branched-chain acyl-CoA and reduction of NAD+ to NADH: R1R2CHCOCOOH + NAD+ + CoASH = R1R2CHCO-S-CoA + NADH + CO2, component E1 (EC 1.2.4.4, branched-chain keto acid dehydrogenase), component E2 (no EC number, branched-chain acyltransferase), component E3 (EC 1.8.1.4, dihydrolipoamide dehydrogenase), partial reactions catalyzed by the multienzyme complex: 1. R1R2CHCOCOOH + E1-(thiamine diphosphate) = E1-(thiamine diphosphate)-COHCHR1R2 + CO2, 2. E1-(thiamine diphosphate)-COHCHR1R2 + lipoyl-E2 = R1R2CHCO-SCoA + dihydrolipoyl-E2, 4. dihydrolipoyl-E2 + NAD+ = lipoyl-E2 + NADH
NAD+
-
the multienzyme complex catalyzes a thiamine diphosphate- and Mg2+-dependent oxidative decarboxylation of branched-chain keto acids with the formation of branched-chain acyl-CoA and reduction of NAD+ to NADH: R1R2CHCOCOOH + NAD+ + CoASH = R1R2CHCO-S-CoA + NADH + CO2, component E1 (EC 1.2.4.4, branched-chain keto acid dehydrogenase), component E2 (no EC number, branched-chain acyltransferase), component E3 (EC 1.8.1.4, dihydrolipoamide dehydrogenase), partial reactions catalyzed by the multienzyme complex: 1. R1R2CHCOCOOH + E1-(thiamine diphosphate) = E1-(thiamine diphosphate)-COHCHR1R2 + CO2, 2. E1-(thiamine diphosphate)-COHCHR1R2 + lipoyl-E2 = R1R2CHCO-SCoA + dihydrolipoyl-E2, 4. dihydrolipoyl-E2 + NAD+ = lipoyl-E2 + NADH
NAD+
-
Km: 0.109 mM in the reaction with 4-methyl-2-oxopentanoate, Km: 0.126 mM in the reaction with 3-methyl-2-oxobutanoate, Km: 0.101 mM in the reaction with DL-3-methyl-2-oxobutanoate
thiamine diphosphate
-
-
thiamine diphosphate
-
dependent
thiamine diphosphate
-
bound to complex component E1, involved in substrate recognition
thiamine diphosphate
-
bound to component E1, heterotetrameric cofactor binding fold, cofactor binding prevents phosphorylation of E1b and inactivates it by inducing a disorder-to-order transition of the conserved phosphorylation loop carrying 2 phosphorylation sites Ser292alpha andSer302alpha, cross-talk between thiamine diphosphate and the phosphorylation loop conformation as a feed-forward switch for th complex reaction
thiamine diphosphate
-
bound to E1b component, binding kinetics, wild-type and mutant E1b components
thiamine diphosphate
-
bound to E1b component, dissociation constants of recombinant wild-type and mutant E1b components