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
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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
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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
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glutarylation
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sylvian
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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
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Natural Substrates Products
Natural Substrates Products on EC 1.3.8.6 - glutaryl-CoA dehydrogenase (ETF)
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REACTION DIAGRAM
glutaryl-CoA + acceptor
crotonoyl-CoA + CO2 + reduced acceptor
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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
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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
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glutaryl-CoA + electron transfer protein
crotonoyl-CoA + CO2 + reduced electron transfer protein
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?
glutaryl-CoA + electron-transfer flavoprotein
crotonyl-CoA + CO2 + reduced electron-transfer flavoprotein
overall reaction
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glutaryl-CoA + acceptor
crotonyl-CoA + CO2 + reduced acceptor
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involved in mitochondrial degradation of lysine, hydroxylysine, tryptophan
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?
glutaryl-CoA + acceptor
crotonyl-CoA + CO2 + reduced acceptor
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analysis of mutations causing glutaric acidemia type I
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glutaryl-CoA + acceptor
crotonyl-CoA + CO2 + reduced acceptor
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deficiency causes glutaric aciduria type I, study on activities in wild type and mutants
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?
(E)-but-2-enoyl-CoA + CO2 + reduced electron transfer flavoprotein
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glutaryl-CoA + electron transfer flavoprotein
(E)-but-2-enoyl-CoA + CO2 + reduced electron transfer flavoprotein
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?
glutaryl-CoA + electron transfer flavoprotein
(E)-but-2-enoyl-CoA + CO2 + reduced electron transfer flavoprotein
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?
glutaryl-CoA + FAD
(E)-but-2-enoyl-CoA + CO2 + FADH2
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GCD catalyzes the oxidative decarboxylation of the gamma-carboxylate of the substrate, glutaryl-CoA, to yield crotonyl-CoA and CO2.
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glutaryl-CoA + FAD
(E)-but-2-enoyl-CoA + CO2 + FADH2
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crotonoyl-CoA + CO2 + FADH2
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via intermediate glutaconyl-CoA
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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
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additional information
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GCDH is a central enzyme in the catabolic pathway of L-tryptophan, L-lysine, and L-hydroxylysine
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additional information
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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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