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Glutathione synthetase is an enzyme crucial for the biosynthesis of glutathione, a tripeptide essential for cellular redox balance and detoxification. It catalyzes the second and final step in glutathione synthesis, joining gamma-glutamylcysteine with glycine in an ATP-dependent reaction. Deficiency of this enzyme leads to low intracellular levels of reduced glutathione and accumulation of its precursor dipeptide gamma-Glu-Cys, resulting in hemolytic anemia, metabolic acidosis, neurological symptoms, and increased susceptibility to oxidative damage due to impaired detoxification capacity.
Acyl phosphate intermediate formation followed by nucleophilic attack
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