Tags: Protein.
Glutathione synthetase (GSS) (EC 6.3.2.3) is the second enzyme in the glutathione biosynthesis pathway. It catalyses the condensation of gamma-glutamylcysteine and glycine to form glutathione.In eukaryotes this is a homodimeric enzyme. 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.