Background: GSS (Glutathione synthetase) is a 474 amino acid protein encoded by the gene located at human chromosome 20q11.2. GSS consists of three loops projecting from an antiparallel β-sheet, a parallel β-sheet and a lid of anti-parallel sheets, which provide access to the ATP-binding site. Although Southern blot and gene analysis suggest that GSS may be the only member of a unique family, the crystal structure indicates that GSS belongs to the ATP-GRASP superfamily. GSS is expressed in hemocytes and nucleated cells, including the brain. GSS occurs as a homodimer. There are two steps in the production of Glutathione, begining with γ-GCS and ending with GSS. In an ATP-dependent reaction, GSS produces Glutathione from γ-glutamylcysteine and glycine precursors. Partial hepatectomy, diethyl maleate, buthionine sulfoximine, tert-butylhaydroquinone and thioacetamide increase the ex-pression of GSS, which causes an increase in Glutathione levels. An inherited autosomal recessive disorder, 5-oxoprolinuria (pyroglutamic aciduria), is caused by GSS deficiencies, which leads to central nervous system damage, hemolytic anemia, metabolic acidosis and urinary excretion of 5-oxoproline. A missense mutation in the gene encoding GSS leads to a GSS deficiency restricted to erythrocytes, which causes only hemolytic anemia.
Description: Rabbit polyclonal to GSS
Immunogen: KLH conjugated synthetic peptide derived from GSS
Specificity: ·Reacts with Human, Mouse and Rat.
·Isotype: IgG
Application: ·Western blotting: 1/100-500. Predicted Mol wt: 52 kDa;
·Immunohistochemistry (Paraffin/frozen tissue section): 1/50-200;
·Immunocytochemistry/Immunofluorescence: 1/100;
·Immunoprecipitation: 1/50;
·ELISA: 1/500;
·Optimal working dilutions must be determined by the end user.