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产品名称:Anti-SGSH/Sulphamidase巨额让利,磺氨基葡糖硫酸胺酶抗体
中文名称:磺氨基葡糖硫酸胺酶抗体
英文名称:Anti-SGSH/Sulphamidase
产品编号:BYK-11756R
产品别名:Heparan sulfate sulfatase; Heparan sulphate sulphatase; HSS; MPS 3A; MPS3 A; MPS3A; N sulfoglucosamine sulfohydrolase (sulfamidase); N-sulphoglucosamine sulphohydrolase; SFMD; SGSH; SPHM_HUMAN; Sulfoglucosamine sulfamidase; Sulphamidase; Sulphoglucosamine sulphamidase.
本公司另供应“磺氨基葡糖硫酸胺酶抗体”的标记有:Alexa Fluor 350 Alexa Fluor 488 Alexa Fluor 555 Alexa Fluor 647 AP APC Biotin Cy3 Cy5 Cy5.5 Cy7 FITC Gold HRP PE PE-Cy3 PE-CY5 PE-CY5.5 PE-CY7 RBITC.
产品规格:100ug/200ug
产品浓度: 1mg/1ml
抗体来源:兔源、鼠源、其他
克 隆:单克隆抗体、多克隆抗体
抗体类型:一抗
性 状: 冻干粉或液体
保存条件: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.
Anti-SGSH/Sulphamidase巨额让利,磺氨基葡糖硫酸胺酶抗体技术说明,产品*,由于篇幅原因,更多信息请:
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产品介绍:
Sulfatases are enzymes that hydrolyse a diverse range of sulfate esters. Deficiency of lysosomal sulfatases leads to human diseases characterized by the accumulation of either GAGs (glycosaminoglycans) or sulfolipids. Sulfamidase, also known as HSS, SFMD, MPS3A or SGSH, is a 502 amino acid lysosome that belongs to the sulfatase family. It has been suggested that sulfamidase may be involved in the lysosomal degradation of heparan sulfate. Defects in the gene encoding sulfamidase are the cause of Sanfilippo syndrome A, an autosomal recessive lysosomal storage disease caused by impaired degradation of heparan sulfate. Sanfilippo syndrome A is characterized by severe central nervous system degeneration but relatively mild somatic manifestations.