Human Glutathione Synthetase, GSS ELI

Glutathione synthetase (GSS) (EC 6.3.2.3) is the second enzyme in the glutathione (GSH) biosynthesis pathway.

It catalyses the condensation of gamma-glutamylcysteine and glycine, to form glutathione.

Glutathione synthetase is also a potent antioxidant.

It is found in many species including bacteria, yeast, mammals, and plants.

In humans, defects in GSS are inherited in an autosomal recessive way and are the cause of severe metabolic acidosis, 5-oxoprolinuria, increased rate of haemolysis, and defective function of the central nervous system.

Deficiencies in GSS can cause a spectrum of deleterious symptoms in plants and human beings alike.