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
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medicine
pharmacology
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altered expression levels in colorectal cancer, which is mainly associated with fatty acid metabolic pathways, speculated to have an important role linked to carcinogenesis
medicine
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glutaric acid, 3-hydroxyglutric acid, and glutarylcarnithine accumulate in patients with GCDH deficiency
medicine
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glutaryl-CoA dehydrogenase deficiency (glutaric acidaemia or aciduria type I) is an autosomal recessive disease which is characterized by an accumulation of glutaric acid, 3-hydroxyglutaric acid, glutaconic acid (less frequently), and glutarylcarnitine in body fluids and tissues
medicine
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glutaryl-CoA dehydrogenase deficiency (glutaric acidemia type 1, GA1) is a disorder of lysine and tryptophan degradation that results in accumulation of glutaric acid and 3-hydroxyglutaric acid in the brain and other tissues
medicine
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glutaryl-CoA dehydrogenase deficiency is an autosomal recessive disease characterized by the accumulation of glutaric and 3-hydroxyglutaric acids in tissues and body fluids causing uncompetitive inhibition of alpha-ketoglutarate dehydrogenase complex
medicine
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glutaryl-CoA dehydrogenase deficiency leads to strong glutaric acid and 3-hydroxyglutaric acid accumulation in the central nervous system
medicine
studies of 18 missense mutations identified in glutaric aciduria type 1 patients affecting surface amino acids. The stability of half of the GCDH mutants is significantly reduced. None of the mutations impairs the 3D structure of GCDH. All GCDH mutants are correctly translocated into mitochondria
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targeted suppression of GCDH by lentivirus-mediated shRNA and excessive intake of lysine may be a useful cell model of glutaric aciduria type 1, overview
pharmacology
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targeted suppression of GCDH by lentivirus-mediated shRNA and excessive intake of lysine may be a useful cell model of glutaric aciduria type 1, overview
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