Glutathione synthase domain (IPR014709)

Short name: Glutathione_synthase_dom

Domain relationships



This entry represents a domain found mainly in eukaryotic glutathione synthetase (EC: (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 [PMID: 15981742]. 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.

Contributing signatures

Signatures from InterPro member databases are used to construct an entry.