Glutathione synthase, substrate-binding domain (IPR004887)

Short name: GSH_synth_subst-bd

Overlapping homologous superfamilies

Domain relationships



This entry represents the substrate-binding domain of 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. The substrate-binding domain has a 3-layer alpha/beta/alpha structure [PMID: 10369661].

GO terms

Biological Process

GO:0006750 glutathione biosynthetic process

Molecular Function

GO:0005524 ATP binding
GO:0004363 glutathione synthase activity

Cellular Component

No terms assigned in this category.

Contributing signatures

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