FAQ

glutaryl-CoA dehydrogenase activity

Known as: glutaryl coenzyme A dehydrogenase activity, glutaryl-CoA:(acceptor) 2,3-oxidoreductase (decarboxylating), glutaryl-CoA:acceptor 2,3-oxidoreductase (decarboxylating) 
Catalysis of the reaction: glutaryl-CoA + acceptor = crotonoyl-CoA + CO2 + reduced acceptor. [EC:1.3.99.7]
National Institutes of Health

Topic mentions per year

Topic mentions per year

1975-2018
01219752018

Papers overview

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2012
2012
Marked reduction of Na(+), K(+)-ATPase and creatine kinase activities induced by acute lysine administration in glutaryl-CoA dehydrogenase deficient mice.
Glutaric acidemia type I (GA I) is an inherited neurometabolic disorder caused by a severe deficiency of the mitochondrial… (More)
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2003
2003
Case Report: Severe phenotype despite high residual glutaryl-CoA dehydrogenase activity: A novel mutation in a Turkish patient with glutaric aciduria type I
We report the first patient with the homozygous GCDH mutation M263V, displaying a high residual activity in fibroblasts of 30… (More)
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1999
1999
Biochemistry of glutaric aciduria type I: Activities of in vitro expressed wild-type and mutant cDNA encoding human glutaryl-CoA dehydrogenase
Glutaric aciduria type I (GA I ; McKusick 231670) is caused by deÐciency of glutaryl-CoA dehydrogenase activity (EC 1.3.99.7), a… (More)
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1998
1998
Glutaric aciduria type I with high residual glutaryl-CoA dehydrogenase activity.
Two brothers with dystonia and slight MRI changes in the basal ganglia had normal urinary glutaric acid excretion, but slightly… (More)
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1993
1993
A fibroblast glutaryl-CoA dehydrogenase assay using detritiation of 3H-labelled glutaryl-CoA: application in the genotyping of the glutaryl-CoA dehydrogenase locus.
A method described earlier for measuring glutaryl-CoA dehydrogenase activity in fibroblasts has been further developed. This… (More)
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1990
1990
Vigabatrin in the treatment of glutaric aciduria type I
Gtutaric aciduria type I is a rare inborn error in the degradation of tysine, hydroxylysine and tryptophan due to a deficiency of… (More)
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1988
1988
Conservation of central nervous system glutaryl-coenzyme A dehydrogenase in fruit-eating bats with glutaric aciduria and deficient hepatic glutaryl-coenzyme A dehydrogenase.
The adult fruit-eating bat, Rousettus aegypticus, excretes massive amounts of glutaric acid in the urine (20-70 mumol/mg… (More)
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1984
1984
Glutaric Aciduria Type II: Evidence for a Defect Related to the Electron Transfer Flavoprotein or Its Dehydrogenase
Summary: Incubation of intact fibroblasts from a patient with glutaric aciduria type II with [2-14C]riboflavin showed normal… (More)
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1980
1980
Glutaric acidemia: a metabolic disorder causing progressive choreoathetosis.
A boy with glutaric acidemia had psychomotor retardation first noted at age 6 months, recurrent metabolic acidosis, and a… (More)
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1975
1975
Glutaric aciduria: inherited deficiency of glutaryl-CoA dehydrogenase activity.
 
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