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Product Name Anti-Glutathione Synthetase/PE-Cy5 Chinese Name PE-Cy5标记的谷胱甘肽合成酶抗体 Alias Glutathione synthase; GSH S; GSH synthetase; GSH-S; GSHB_HUMAN; GSHS; GSS; MGC14098; OTTHUMP00000030711. Research Area Tumour Cell biology Neurobiology Signal transduction The new supersedes the old Immunogen Species Rabbit Clonality Polyclonal React Species (predicted: Human, Mouse, Rat, Dog, Pig, Cow, Horse, Sheep, ) Applications ICC=1:50-200 IF=1:50-200
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.Molecular weight 52kDa Form Lyophilized or Liquid Concentration 1mg/ml immunogen KLH conjugated synthetic peptide derived from human Glutathione Synthetase Lsotype IgG Purification affinity purified by Protein A Storage Buffer 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol. Storage Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. The lyophilized antibody is stable at room temperature for at least one month and for greater than a year when kept at -20°C. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 °C. Product Detail 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 ∫-sheet, a parallel ∫-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 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 ∫-sheet, a parallel ∫-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 ©-GCS and ending with GSS. In an ATP-dependent reaction, GSS produces Glutathione from ©-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.-GCS and ending with GSS. In an ATP-dependent reaction, GSS produces Glutathione from ©-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.
Function:
Sulfur metabolism; glutathione biosynthesis; glutathione from L-cysteine and L-glutamate: step 2/2.
Subunit:
Homodimer.
DISEASE:
Defects in GSS are the cause of glutathione synthetase deficiency (GSS deficiency) [MIM:266130]; 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.
Similarity:
Belongs to the eukaryotic GSH synthase family.
Database links:Entrez Gene: 2937 Human
Entrez Gene: 14854 Mouse
Omim: 601002 Human
SwissProt: P48637 Human
SwissProt: P51855 Mouse
Unigene: 82327 Human
Unigene: 252316 Mouse
Unigene: 1692 Rat
Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
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