Catalyzes the production of glutathione from gamma-glutamylcysteine and glycine in an ATP-dependent manner (PubMed:7646467, PubMed:9215686). Glutathione (gamma-glutamylcysteinylglycine, GSH) is the most abundant intracellular thiol in living aerobic cells and is required for numerous processes including the protection of cells against oxidative damage, amino acid transport, the detoxification of foreign compounds, the maintenance of protein sulfhydryl groups in a reduced state and acts as a cofactor for a number of enzymes (PubMed:10369661). Participates in ophthalmate biosynthesis in hepatocytes (By similarity).
Glutathione synthetase deficiency
GSS deficiency
Severe form characterized by an increased rate of hemolysis and defective function of the central nervous system.
None
The disease is caused by variants affecting the gene represented in this entry.
Glutathione synthetase deficiency of erythrocytes
GLUSYNDE
Mild form causing hemolytic anemia.
None
The disease is caused by variants affecting the gene represented in this entry.
Sulfur metabolism; glutathione biosynthesis; glutathione from L-cysteine and L-glutamate: step 2/2.
Belongs to the eukaryotic GSH synthase family.
Glutathione synthetase, GSH synthetase, GSH-S, Glutathione synthase, GSS
Proteins
Oncology
52385Da
We found 2 products in 1 category
ab178385