InterPro domain: IPR037013

This superfamily represents the substrate-binding domain of 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 substrate-binding domain has a 3-layer alpha/beta/alpha structure [ 2 ].

1. Physiological and pathological aspects of GSH metabolism. Acta Paediatr. 94, 132-7
2. Molecular basis of glutathione synthetase deficiency and a rare gene permutation event. EMBO J. 18, 3204-13