<p>This entry represents the substrate-binding domain of glutathione synthetase (<db_xref db="EC" dbkey="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 [<cite idref="PUB00035960"/>]. 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 [<cite idref="PUB00019656"/>].</p> Glutathione synthase, substrate-binding, eukaryotic