1.3.8.6: glutaryl-CoA dehydrogenase (ETF)
This is an abbreviated version!
For detailed information about glutaryl-CoA dehydrogenase (ETF), go to the full flat file.
Word Map on EC 1.3.8.6
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1.3.8.6
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glutaric
-
aciduria
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3-hydroxyglutaric
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striatal
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dystonia
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encephalopathic
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ga1
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macrocephaly
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inborn
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neurometabolic
-
crises
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glutarylcarnitine
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hydroxylysine
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carnitine
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crotonyl-coa
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glutaconic
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intercurrent
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3-oh-ga
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frontotemporal
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neuroradiological
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acylcarnitine
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excretors
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medicine
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glutaconyl-coa
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acidaemia
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subdural
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isovaleryl-coa
-
glutarylation
-
sylvian
-
pharmacology
- 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
Reaction
Synonyms
decarboxylating glutaryl-coenzyme A dehydrogenase, EC 1.3.99.7, GCD, GCDH, GDH, GDHGeo, glutaryl coenzyme A dehydrogenase, glutaryl-CoA dehydrogenase, glutaryl-coenzyme A dehydrogenase, More
ECTree
1.3.8.6 glutaryl-CoA dehydrogenase (ETF)Advanced search results
results (
results (Substrates Products
Substrates Products on EC 1.3.8.6 - glutaryl-CoA dehydrogenase (ETF)
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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
-
-
?
