| glutaryl |
the divalent radical of glutaric acid; as glutaryl CoA, a thioester formed with coenzyme A, it is an intermediate in the catabolism of lysine, hydroxylysine, and tryptophan.
출처: www.mercksource.com/pp/us/cns/cns_health_library.j...
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| glutaryl-CoA dehydrogenase |
[EC 1.3.99.7] an enzyme of the oxidoreductase class that catalyzes the oxidative decarboxylation of glutaryl CoA, with sequential reduction of FAD and then electron transfer flavoprotein; the reaction is a step in the degradation of lysine, hydroxylysine, and tryptophan. Deficiency of the enzyme, an autosomal recessive trait, causes glutaricaciduria, type I.
출처: www.mercksource.com/pp/us/cns/cns_health_library.j...
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| glutathione (GSH) synthetase deficiency |
an autosomal recessive aminoacidopathy due to decreased levels of GSH and increased levels of 5-oxoproline and cysteine, occurring in two phenotypes. Deficiency of glutathione synthetase confined to the erythrocytes results in a well-compensated hemolytic anemia; generalized deficiency of the enzyme causes high levels of 5-oxoproline in plasma and urine, metabolic acidosis, and often neurologic dysfunction, along with hemolytic anemia.
출처: www.mercksource.com/pp/us/cns/cns_health_library.j...
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| glutathione reductase (NADPH) |
[EC 1.6.4.2] an enzyme of the oxidoreductase class that catalyzes the reduction of glutathione via oxidation of NADPH. It is a flavoprotein (FAD), occurring in erythrocytes, and is involved in many redox reactions. Deficiency of enzyme activity in erythrocytes usually results from nutritional or metabolic inadequacy of FAD and, except when severe, has not been linked to hemolysis. Diminished enzyme activity does decrease protection of cells from oxidative damage.
출처: www.mercksource.com/pp/us/cns/cns_health_library.j...
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| glutathione synthase |
[EC 6.3.2.3] glutathione synthetase.
출처: www.mercksource.com/pp/us/cns/cns_health_library.j...
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