| 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.
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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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| glutathione synthetase |
an enzyme of the ligase class that catalyzes the ATP-dependent formation of glutathione from glycine and γ-glutamylcysteine. Deficiency of the enzyme, an autosomal recessive trait, alters the γ-glutamyl cycle; γ´-glutamylcysteine synthesized futilely is converted to excess 5-oxoproline and cysteine. In EC nomenclature, called glutathione synthase.
출처: www.mercksource.com/pp/us/cns/cns_health_library.j...
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| glutathionemia |
the presence of glutathione in the blood.
출처: www.mercksource.com/pp/us/cns/cns_health_library.j...
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