glutathione synthetase


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glu·ta·thi·one syn·the·tase

[MIM*601002]
an enzyme that catalyzes the formation of glutathione, ADP, and orthophosphate from γ-glutamylcysteine, ATP, and glycine; a deficiency will lead to metabolic acidosis and progressive brain dysfunction.

glutathione synthetase

/glu·ta·thi·one syn·the·tase/ (sin´thĕ-tās) a ligase catalyzing the formation of glutathione; deficient activity causes decreased levels of glutathione and increased levels of 5-oxoproline and cysteine. If confined to erythrocytes, the deficiency results in well-compensated hemolytic anemia; if generalized, metabolic acidosis and neurologic dysfunction may also occur.

glutathione synthetase

One of the enzymes concerned in the synthesis of GLUTATHIONE from glutamate.
References in periodicals archive ?
Two genetic deficiencies of the [gamma]-glutamyl cycle, glutathione synthetase deficiency and 5-oxoprolinase deficiency (3), lead to very large increases in 5-oxoproline excretion.
Finally, a previous investigation of another subject with acute onset 5-oxoprolinuria yielded normal activities of glutathione synthetase and 5-oxoprolinase (1).
However, it is possible that, under the altered conditions resulting from acetaminophen ingestion, glutathione synthetase becomes rate limiting or is inhibited, leading to the accumulation of [gamma]-glutamyl cysteine, which can then be acted on by [gamma]-glutamyl cyclotransferase to produce L-5-oxoproline (Fig.
An inherited defect in the enzyme glutathione synthetase was first described in a 19-year-old man in 1970 [3].
The only main abnormality evident in affected individuals is increased concentrations of 5-oxoproline, which are not so great as those found in cases of glutathione synthetase deficiency and characteristically do not result in major metabolic acidosis.