谷胱甘肽合成酶抗体
规格：1尘驳/1尘濒
英文名： Glutathione Synthetase
别名： Glutathione synthase; GSH S; GSH synthetase; GSH-S; GSHB_HUMAN; GSHS; GSS; MGC14098; OTTHUMP00000030711.
分子量： 52kDa
储存液：0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glyce
克隆类型：笔辞濒测肠濒辞苍补濒
亚型：滨驳骋
纯化方法：affinity purified by Protein A
**原：KLH conjugated synthetic peptide derived from human Glutathi
交叉反应：Human, Mouse, Rat, Dog, Pig, Cow, Horse, Sheep,
细胞定位：
谷胱甘肽合成酶抗体产物介绍：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,谷胱甘肽合成酶抗体 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 谷胱甘肽合成酶抗体(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. Gene ID: 2937 Database links: Entrez Gene: 2937 Human Entrez Gene: 14854 Mouse Entrez Gene: 25458 Rat Omim: 601002 Human SwissProt: P48637 Human SwissProt: P51855 Mouse SwissProt: P46413 Rat 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.
谷胱甘肽合成酶抗体产物应用：WB=1:100-500 ELISA=1:500-1000 IHC-P=1:100-500 IHC-F=1:100-500 ICC=1:100-500 IF=1:100-500 （石蜡切片需做抗原修复） not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user.
研究领域：肿瘤 细胞生物 神经生物学 信号转导 新陈代谢
储存条件： Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
来源： Rabbit
外观： Lyophilized or Liquid