| Primary Identifier | IPR014042 | Type | Homologous_superfamily |
| Short Name | Glutathione_synthase_a-hlx |
| description | This superfamily represents an α-helical domain found in 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. |
