Type: | Domain | Name: | Glutathione synthase domain |
Description: | This entry represents a domain found mainly in eukaryotic glutathione synthetase () (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 []. 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.This domain has a 2-layer alpha/beta structure. | Short Name: | Glutathione_synthase_dom |