Rabbit GSS Polyclonal Antibody | anti-GSS antibody
GSS Polyclonal Antibody
Aliquot and store at -20 degree C long term.
Avoid freeze-thaw cycles.
NCBI and Uniprot Product Information
NCBI Description
Glutathione is important for a variety of biological functions, including protection of cells from oxidative damage by free radicals, detoxification of xenobiotics, and membrane transport. The protein encoded by this gene functions as a homodimer to catalyze the second step of glutathione biosynthesis, which is the ATP-dependent conversion of gamma-L-glutamyl-L-cysteine to glutathione. Defects in this gene are a cause of glutathione synthetase deficiency. [provided by RefSeq, Jul 2008]
Uniprot Description
GSS: Defects in GSS are the cause of glutathione synthetase deficiency (GSS deficiency); also known as 5- oxoprolinuria or pyroglutamic aciduria. It is a severe form characterized by an increased rate of hemolysis and defective function of the central nervous system. Defects in GSS are the cause of glutathione synthetase deficiency of erythrocytes (GLUSYNDE)[MIM:231900]. Glutathione synthetase deficiency of erythrocytes is a mild form causing hemolytic anemia. Belongs to the eukaryotic GSH synthase family.
Protein type: EC 6.3.2.3; Other Amino Acids Metabolism - glutathione; Ligase
Chromosomal Location of Human Ortholog: 20q11.2
Cellular Component: cytosol
Molecular Function: protein homodimerization activity; glutathione binding; glycine binding; magnesium ion binding; glutathione synthase activity; ATP binding
Biological Process: amino acid metabolic process; response to nutrient levels; nervous system development; response to cadmium ion; xenobiotic metabolic process; glutathione biosynthetic process; response to oxidative stress; response to amino acid stimulus; aging
Disease: Glutathione Synthetase Deficiency; Glutathione Synthetase Deficiency Of Erythrocytes, Hemolytic Anemia Due To