InterPro domain: IPR014049

This superfamily represents the N-terminal domain found in eukaryotic glutathione synthetase ( 6.3.2.3 ) (GSS), a homodimeric enzyme that catalyses the conversion of gamma-L-glutamyl-L-cysteine and glycine to phosphate and glutathione in the presence of ATP. This is the second step in glutathione biosynthesis, the first step being catalysed by gamma-glutamylcysteine synthetase [ 1 ]. In humans, defects in GSS are inherited in an autosomal recessive way and are the cause of severe metabolic acidosis, 5-oxoprolinuria, and increased rate of haemolysis and defective function of the central nervous system. The N-terminal domain has a 2-layer alpha/beta structure.

1. Physiological and pathological aspects of GSH metabolism. Acta Paediatr. 94, 132-7