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Description
Moderate glutathione synthetase deficiency leads to hemolytic anemia and metabolic acidosis, whereas patients with a more severe deficiency also develop 5-oxoprolinuria, recurrent bacterial infections, and progressive dysfunction of the central nervous system, including mental retardation and motor functional disturbances
[PubMed ID: 3015905]
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Therefore, the molecular mechanisms underlying GSH dysfunction in the brain differ from those in peripheral tissues
