Domain

Glutathione synthase, alpha-helical, eukaryotic (IPR014042)

Short name: Glutathione_synthase_a-hlx_euk

Domain relationships

None.

Description

This entry represents an alpha-helical domain found in eukaryotic glutathione synthetase (EC:6.3.2.3) (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.

GO terms

Biological Process

GO:0006750 glutathione biosynthetic process

Molecular Function

GO:0005524 ATP binding
GO:0004363 glutathione synthase activity
GO:0016874 ligase activity

Cellular Component

No terms assigned in this category.

Contributing signatures

Signatures from InterPro member databases are used to construct an entry.
GENE3D