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Information on EC 1.3.8.6 - glutaryl-CoA dehydrogenase (ETF)
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EC Tree
1.3.8.6 glutaryl-CoA dehydrogenase (ETF)IUBMB Comments
Contains FAD. The enzyme catalyses the oxidation of glutaryl-CoA to glutaconyl-CoA (which remains bound to the enzyme), and the decarboxylation of the latter to crotonyl-CoA (cf. EC 7.2.4.5, glutaconyl-CoA decarboxylase). FAD is the electron acceptor in the oxidation of the substrate, and its reoxidation by electron-transfer flavoprotein completes the catalytic cycle. The anaerobic, sulfate-reducing bacterium Desulfococcus multivorans contains two glutaryl-CoA dehydrogenases: a decarboxylating enzyme (this entry), and a non-decarboxylating enzyme that only catalyses the oxidation to glutaconyl-CoA [EC 1.3.99.32, glutaryl-CoA dehydrogenase (acceptor)].
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Word Map
- 1.3.8.6
-
glutaric
- aciduria
-
3-hydroxyglutaric
- striatal
- dystonia
-
encephalopathic
- ga1
-
macrocephaly
-
inborn
-
neurometabolic
- crises
-
glutarylcarnitine
- hydroxylysine
- carnitine
- crotonyl-coa
-
glutaconic
-
intercurrent
-
3-oh-ga
-
frontotemporal
-
neuroradiological
- acylcarnitine
-
excretors
- medicine
- glutaconyl-coa
-
acidaemia
-
subdural
- isovaleryl-coa
-
glutarylation
-
sylvian
- pharmacology
The expected taxonomic range for this enzyme is: Bacteria, Archaea, Eukaryota
Reaction Schemes
+
=
+
+
Synonyms
glutaryl-coa dehydrogenase, glutaryl-coenzyme a dehydrogenase, gdhgeo, glutaryl coenzyme a dehydrogenase, 
more
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SYNONYM 
ORGANISM
UNIPROT
COMMENTARY 
LITERATURE
EC 1.3.99.7
-
-
formerly
-
GCD
GCDH
GDH
glutaryl coenzyme A dehydrogenase
glutaryl-CoA dehydrogenase
glutaryl-coenzyme A dehydrogenase
additional information
enzyme belongs to the acyl-CoA dehydrogenase family of enzymes
GCD
-
-
-
-
glutaryl coenzyme A dehydrogenase
-
-
-
-
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REACTION
REACTION DIAGRAM
COMMENTARY
ORGANISM
UNIPROT
LITERATURE
(E)-glutaconyl-CoA = crotonyl-CoA + CO2
(1b)
-
-
-
glutaryl-CoA + electron-transfer flavoprotein = (E)-glutaconyl-CoA + reduced electron-transfer flavoprotein
(1a)
-
-
-
glutaryl-CoA + electron-transfer flavoprotein = crotonyl-CoA + CO2 + reduced electron-transfer flavoprotein
glutaryl-CoA + electron-transfer flavoprotein = crotonyl-CoA + CO2 + reduced electron-transfer flavoprotein
-
-
-
-
glutaryl-CoA + electron-transfer flavoprotein = crotonyl-CoA + CO2 + reduced electron-transfer flavoprotein
structural bases and mechanism of dehydrogenation and decarboxylation reactions, active site structure with and without bound substrate, Glu370 is important
glutaryl-CoA + electron-transfer flavoprotein = crotonyl-CoA + CO2 + reduced electron-transfer flavoprotein
overall reaction
-
-
-
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REACTION TYPE
ORGANISM
UNIPROT
COMMENTARY
LITERATURE
decarboxylation
dehydrogenation
-
-
-
-
oxidation
-
-
-
-
redox reaction
-
-
-
-
reduction
-
-
-
-
decarboxylation
-
-
-
-
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PATHWAY SOURCE
PATHWAYS
BRENDA
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SYSTEMATIC NAME
IUBMB Comments
glutaryl-CoA:electron-transfer flavoprotein 2,3-oxidoreductase (decarboxylating)
Contains FAD. The enzyme catalyses the oxidation of glutaryl-CoA to glutaconyl-CoA (which remains bound to the enzyme), and the decarboxylation of the latter to crotonyl-CoA (cf. EC 7.2.4.5, glutaconyl-CoA decarboxylase). FAD is the electron acceptor in the oxidation of the substrate, and its reoxidation by electron-transfer flavoprotein completes the catalytic cycle. The anaerobic, sulfate-reducing bacterium Desulfococcus multivorans contains two glutaryl-CoA dehydrogenases: a decarboxylating enzyme (this entry), and a non-decarboxylating enzyme that only catalyses the oxidation to glutaconyl-CoA [EC 1.3.99.32, glutaryl-CoA dehydrogenase (acceptor)].
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CAS REGISTRY NUMBER
COMMENTARY
37255-38-2
-
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SUBSTRATE
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
4-nitrobutyryl-CoA + electron transfer protein
? + CO2 + reduced electron transfer protein
-
-
-
?
glutaconyl-CoA + ferrocenium hexafluorophosphate
crotonyl-CoA + CO2 + ferricenium hexafluorophosphate
glutaryl-CoA + 2,6-dichlorophenol indophenol
crotonoyl-CoA + CO2 + reduced 2,6-dichlorophenol indophenol
glutaryl-CoA + electron transfer flavoprotein
(E)-but-2-enoyl-CoA + CO2 + reduced electron transfer flavoprotein
glutaryl-CoA + electron transfer flavoprotein
crotonoyl-CoA + CO2 + reduced electron transfer flavoprotein
-
-
-
-
?
glutaryl-CoA + electron transfer protein
crotonoyl-CoA + CO2 + reduced electron transfer protein
-
-
-
?
glutaryl-CoA + electron-transfer flavoprotein
crotonyl-CoA + CO2 + reduced electron-transfer flavoprotein
glutaryl-CoA + ferricenium hexafluorophosphate
crotonyl-CoA + CO2 + ferrocenium hexafluorophosphate
glutaryl-CoA + human electron-transfer flavoprotein
crotonyl-CoA + CO2 + reduced human electron-transfer flavoprotein
-
-
-
-
r
glutaryl-CoA + phenazine methosulfate
crotonyl-CoA + CO2 + reduced phenazine methosulfate
-
-
-
-
r
4-nitrobutyryl-CoA + acceptor
4-nitro-but-2-enoyl-CoA + reduced acceptor
-
-
-
?
4-nitrobutyryl-CoA + acceptor
4-nitro-but-2-enoyl-CoA + reduced acceptor
-
-
-
?
glutaconyl-CoA + acceptor
crotonyl-CoA + CO2 + reduced acceptor
-
involvement of water in catalysis, previously unrecognized and in conflict with a classically held intramolecular 1,3-prototropic shift for protonation of crotonyl-CoA dienolate
-
-
?
glutaconyl-CoA + ferrocenium hexafluorophosphate
crotonyl-CoA + CO2 + ferricenium hexafluorophosphate
-
-
-
-
?
glutaconyl-CoA + ferrocenium hexafluorophosphate
crotonyl-CoA + CO2 + ferricenium hexafluorophosphate
-
the kinetically favorable product is vinylacetyl-CoA, which is further isomerized to the thermodynamically stable normal product crotonyl-CoA
-
ir
glutaryl-CoA + 2,6-dichlorophenol indophenol
crotonoyl-CoA + CO2 + reduced 2,6-dichlorophenol indophenol
-
-
-
-
?
glutaryl-CoA + 2,6-dichlorophenol indophenol
crotonoyl-CoA + CO2 + reduced 2,6-dichlorophenol indophenol
-
-
-
?
glutaryl-CoA + acceptor
crotonoyl-CoA + CO2 + reduced acceptor
-
part of the degradative pathway of the amino acids tryptophan, lysine, and hydroxylysine, enzyme deficiency leads to glutaric aciduria type I leading to nonspecific developmental delay, hypotonia, and macrocephaly with cerebral atrophyof prenatal onset
-
-
?
glutaryl-CoA + acceptor
crotonyl-CoA + CO2 + reduced acceptor
-
acceptors: electron-transfer flavoprotein, phenylmethylsulfonyl fluoride, 2,6-dichlorophenolindophenol, phenazine methosulfate, methylene blue
-
?
glutaryl-CoA + acceptor
crotonyl-CoA + CO2 + reduced acceptor
-
-
-
-
?
glutaryl-CoA + acceptor
crotonyl-CoA + CO2 + reduced acceptor
-
site directed mutagenesis of binding site and analysis of mechanism
-
-
?
glutaryl-CoA + acceptor
crotonyl-CoA + CO2 + reduced acceptor
-
acceptors: electron-transfer flavoprotein, phenylmethylsulfonyl fluoride, 2,6-dichlorophenolindophenol, phenazine methosulfate, methylene blue
-
?
glutaryl-CoA + acceptor
crotonyl-CoA + CO2 + reduced acceptor
-
acceptors: electron-transfer flavoprotein, phenylmethylsulfonyl fluoride, 2,6-dichlorophenolindophenol, phenazine methosulfate, methylene blue
-
?
glutaryl-CoA + acceptor
crotonyl-CoA + CO2 + reduced acceptor
-
analysis of mechanism , redox potential
-
?
glutaryl-CoA + acceptor
crotonyl-CoA + CO2 + reduced acceptor
-
analysis of decarboxylation reaction, enzyme has intrinsic enoyl-CoA hydratase activity
-
-
?
glutaryl-CoA + acceptor
crotonyl-CoA + CO2 + reduced acceptor
-
acceptor: ferrocenium hexafluorophosphate
-
?
glutaryl-CoA + acceptor
crotonyl-CoA + CO2 + reduced acceptor
-
acceptor: ferrocenium hexafluorophosphate
-
-
?
glutaryl-CoA + acceptor
crotonyl-CoA + CO2 + reduced acceptor
-
involved in mitochondrial degradation of lysine, hydroxylysine, tryptophan
-
-
?
glutaryl-CoA + acceptor
crotonyl-CoA + CO2 + reduced acceptor
-
analysis of mutations causing glutaric acidemia type I
-
-
?
glutaryl-CoA + acceptor
crotonyl-CoA + CO2 + reduced acceptor
-
deficiency causes glutaric aciduria type I, study on activities in wild type and mutants
-
?
glutaryl-CoA + acceptor
crotonyl-CoA + CO2 + reduced acceptor
-
inhibition of redox half-reaction in mitochondria leads to oxidation of glutaryl-CoA to glutaconyl-CoA in peroxisomes and its decarboxylation by glutaryl-decarboxylase in mitochondria
-
-
?
glutaryl-CoA + acceptor
crotonyl-CoA + CO2 + reduced acceptor
-
acceptors: electron-transfer flavoprotein, phenylmethylsulfonyl fluoride, 2,6-dichlorophenolindophenol, phenazine methosulfate, methylene blue
-
?
glutaryl-CoA + acceptor
crotonyl-CoA + CO2 + reduced acceptor
-
acceptors: electron-transfer flavoprotein, phenylmethylsulfonyl fluoride, 2,6-dichlorophenolindophenol, phenazine methosulfate, methylene blue
-
?
glutaryl-CoA + acceptor
crotonyl-CoA + CO2 + reduced acceptor
-
acceptors: electron-transfer flavoprotein, phenylmethylsulfonyl fluoride, 2,6-dichlorophenolindophenol, phenazine methosulfate, methylene blue
-
?
glutaryl-CoA + acceptor
crotonyl-CoA + CO2 + reduced acceptor
-
acceptors: electron-transfer flavoprotein, phenylmethylsulfonyl fluoride, 2,6-dichlorophenolindophenol, phenazine methosulfate, methylene blue
-
?
glutaryl-CoA + acceptor
crotonyl-CoA + CO2 + reduced acceptor
-
acceptors: electron-transfer flavoprotein, phenylmethylsulfonyl fluoride, 2,6-dichlorophenolindophenol, phenazine methosulfate, methylene blue
-
?
glutaryl-CoA + acceptor
crotonyl-CoA + CO2 + reduced acceptor
-
acceptors: electron-transfer flavoprotein, phenylmethylsulfonyl fluoride, 2,6-dichlorophenolindophenol, phenazine methosulfate, methylene blue
-
?
glutaryl-CoA + electron transfer flavoprotein
(E)-but-2-enoyl-CoA + CO2 + reduced electron transfer flavoprotein
-
-
-
?
glutaryl-CoA + electron transfer flavoprotein
(E)-but-2-enoyl-CoA + CO2 + reduced electron transfer flavoprotein
-
-
-
?
glutaryl-CoA + electron transfer flavoprotein
(E)-but-2-enoyl-CoA + CO2 + reduced electron transfer flavoprotein
-
-
-
-
?
glutaryl-CoA + electron-transfer flavoprotein
crotonyl-CoA + CO2 + reduced electron-transfer flavoprotein
-
-
-
-
r
glutaryl-CoA + electron-transfer flavoprotein
crotonyl-CoA + CO2 + reduced electron-transfer flavoprotein
overall reaction
-
-
?
glutaryl-CoA + electron-transfer flavoprotein
crotonyl-CoA + CO2 + reduced electron-transfer flavoprotein
-
-
-
-
r
glutaryl-CoA + electron-transfer flavoprotein
crotonyl-CoA + CO2 + reduced electron-transfer flavoprotein
-
-
-
-
r
glutaryl-CoA + electron-transfer flavoprotein
crotonyl-CoA + CO2 + reduced electron-transfer flavoprotein
-
-
-
-
r
glutaryl-CoA + FAD
(E)-but-2-enoyl-CoA + CO2 + FADH2
-
GCD catalyzes the oxidative decarboxylation of the gamma-carboxylate of the substrate, glutaryl-CoA, to yield crotonyl-CoA and CO2.
-
-
?
glutaryl-CoA + FAD
(E)-but-2-enoyl-CoA + CO2 + FADH2
-
-
-
-
?
glutaryl-CoA + FAD
crotonoyl-CoA + CO2 + FADH2
-
via intermediate glutaconyl-CoA
-
-
?
glutaryl-CoA + FAD
crotonoyl-CoA + CO2 + FADH2
-
via intermediate glutaconyl-CoA, detection using ferrocenium hexafluorophosphate
-
-
?
glutaryl-CoA + FAD
crotonoyl-CoA + CO2 + FADH2
detection using ferrocenium hexafluorophosphate
-
-
?
glutaryl-CoA + ferricenium hexafluorophosphate
crotonyl-CoA + CO2 + ferrocenium hexafluorophosphate
-
-
-
?
glutaryl-CoA + ferricenium hexafluorophosphate
crotonyl-CoA + CO2 + ferrocenium hexafluorophosphate
-
-
-
-
?
glutaryl-CoA + ferricenium hexafluorophosphate
crotonyl-CoA + CO2 + ferrocenium hexafluorophosphate
-
-
-
-
?
glutarylpantetheine + acceptor
crotonylpantetheine + reduced acceptor
-
-
-
?
glutarylpantetheine + acceptor
crotonylpantetheine + reduced acceptor
-
-
-
?
additional information
?
-
-
enzyme deficiency leads to glutaric aciduria type I with accumulation of glutarate and 3-hydroxyglutarate with subsequent neuronal destruction during metabolic crises triggered by catabolic stress, treatment with isocaloric glucose and carnitine infusion and suspension of protein intake can recover the patient from apathy
-
-
?
additional information
?
-
-
GCDH is a central enzyme in the catabolic pathway of L-tryptophan, L-lysine, and L-hydroxylysine
-
-
?
additional information
?
-
the decarboxylating and nondecarboxylating, EC 1.3.99.X from Desulfococcus multivorans, capabilities are provided by complex structural changes around the glutaconyl carboxylate group, the key factor being a Tyr to Val exchange strictly conserved between the two GDH types
-
-
?
additional information
?
-
GCDH enzyme activity is measured following the reduction of DCPIP at 600 nm, with either PMS (artificial electron acceptor) or recombinant human ETF (GCDH natural electron acceptor), and glutaryl-CoA as electron donor, in 10 mM HEPES, pH 7.8
-
-
-
additional information
?
-
-
GCDH enzyme activity is measured following the reduction of DCPIP at 600 nm, with either PMS (artificial electron acceptor) or recombinant human ETF (GCDH natural electron acceptor), and glutaryl-CoA as electron donor, in 10 mM HEPES, pH 7.8
-
-
-
additional information
?
-
-
spectral and electrochemical properties
-
-
?
additional information
?
-
-
the enzyme is also active with butyryl-CoA, hexanoyl-CoA, octanoyl-CoA, and decanoyl-CoA, but is inactive with decanoyl-CoA and dodecanoyl-CoA, substrate specificity, overview
-
-
?
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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
glutaryl-CoA + acceptor
crotonoyl-CoA + CO2 + reduced acceptor
-
part of the degradative pathway of the amino acids tryptophan, lysine, and hydroxylysine, enzyme deficiency leads to glutaric aciduria type I leading to nonspecific developmental delay, hypotonia, and macrocephaly with cerebral atrophyof prenatal onset
-
-
?
glutaryl-CoA + electron transfer flavoprotein
(E)-but-2-enoyl-CoA + CO2 + reduced electron transfer flavoprotein
glutaryl-CoA + electron transfer flavoprotein
crotonoyl-CoA + CO2 + reduced electron transfer flavoprotein
-
-
-
-
?
glutaryl-CoA + electron transfer protein
crotonoyl-CoA + CO2 + reduced electron transfer protein
-
-
-
?
glutaryl-CoA + electron-transfer flavoprotein
crotonyl-CoA + CO2 + reduced electron-transfer flavoprotein
overall reaction
-
-
?
glutaryl-CoA + acceptor
crotonyl-CoA + CO2 + reduced acceptor
-
involved in mitochondrial degradation of lysine, hydroxylysine, tryptophan
-
-
?
glutaryl-CoA + acceptor
crotonyl-CoA + CO2 + reduced acceptor
-
analysis of mutations causing glutaric acidemia type I
-
-
?
glutaryl-CoA + acceptor
crotonyl-CoA + CO2 + reduced acceptor
-
deficiency causes glutaric aciduria type I, study on activities in wild type and mutants
-
?
glutaryl-CoA + electron transfer flavoprotein
(E)-but-2-enoyl-CoA + CO2 + reduced electron transfer flavoprotein
-
-
-
?
glutaryl-CoA + electron transfer flavoprotein
(E)-but-2-enoyl-CoA + CO2 + reduced electron transfer flavoprotein
-
-
-
?
glutaryl-CoA + electron transfer flavoprotein
(E)-but-2-enoyl-CoA + CO2 + reduced electron transfer flavoprotein
-
-
-
-
?
glutaryl-CoA + FAD
(E)-but-2-enoyl-CoA + CO2 + FADH2
-
GCD catalyzes the oxidative decarboxylation of the gamma-carboxylate of the substrate, glutaryl-CoA, to yield crotonyl-CoA and CO2.
-
-
?
glutaryl-CoA + FAD
(E)-but-2-enoyl-CoA + CO2 + FADH2
-
-
-
-
?
glutaryl-CoA + FAD
crotonoyl-CoA + CO2 + FADH2
-
via intermediate glutaconyl-CoA
-
-
?
additional information
?
-
-
enzyme deficiency leads to glutaric aciduria type I with accumulation of glutarate and 3-hydroxyglutarate with subsequent neuronal destruction during metabolic crises triggered by catabolic stress, treatment with isocaloric glucose and carnitine infusion and suspension of protein intake can recover the patient from apathy
-
-
?
additional information
?
-
-
GCDH is a central enzyme in the catabolic pathway of L-tryptophan, L-lysine, and L-hydroxylysine
-
-
?
additional information
?
-
the decarboxylating and nondecarboxylating, EC 1.3.99.X from Desulfococcus multivorans, capabilities are provided by complex structural changes around the glutaconyl carboxylate group, the key factor being a Tyr to Val exchange strictly conserved between the two GDH types
-
-
?
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COFACTOR
ORGANISM
UNIPROT
COMMENTARY
LITERATURE
IMAGE
electron transferring flavoprotein
ETF, the GCDH natural electron acceptor
FAD
oxidation of reduced FAD is much faster than the chemical steps that occur in the active site of the enzyme
additional information
electron acceptor: 2,6-dichlorophenol indophenol
-
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INHIBITOR
ORGANISM
UNIPROT
COMMENTARY
LITERATURE
IMAGE
3-thiaglutaryl-CoA
non-oxidizable analogue, which competes with 2-pentynoyl-CoA for the binding site
oct-2-yn-4-enoyl-CoA
-
irreversible and most likely involved covalent modification of the apoenzyme
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ACTIVATING COMPOUND
ORGANISM
UNIPROT
COMMENTARY
LITERATURE
IMAGE
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KM VALUE [mM]
SUBSTRATE
ORGANISM
UNIPROT
COMMENTARY
LITERATURE
IMAGE
0.0004
electron transfer flavoprotein
-
recombinant wild-type enzyme, pH 7.6, 25°C
0.0064
glutaryl-CoA
-
recombinant wild-type enzyme, pH 7.6, 25°C, electron acceptor is electron transfer flavoprotein
0.0074
glutaryl-CoA
-
mutant A421T, pH 7.6, 25°C, electron acceptor is electron transfer flavoprotein
0.0123
glutaryl-CoA
-
mutant A421V, pH 7.6, 25°C, electron acceptor is electron transfer flavoprotein
0.02
glutaryl-CoA
recombinant mutant V400M with ETF, pH 7.8, temperature not specified in the publication
0.0211
glutaryl-CoA
recombinant wild-type enzyme with ETF, pH 7.8, temperature not specified in the publication
0.0261
glutaryl-CoA
-
mutant A433V, pH 7.6, 25°C, electron acceptor is electron transfer flavoprotein
0.0326
glutaryl-CoA
recombinant mutant R227P with ETF, pH 7.8, temperature not specified in the publication
0.035
glutaryl-CoA
-
mutant E370Q, pH and temperature not specified in the publication
0.0481
glutaryl-CoA