β葡萄糖醛酸苷酶抗体

参考价:¥1
供货周期: 现货
品牌: CST
规格: g/mg
货号: hz-7980R
CAS号:
上海沪震生物科技有限公司
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产品详情

英文名称    beta glucuronidase    

中文名称    β葡萄糖醛酸苷酶抗体    

别    名    asd; Beta G1; Beta glucuronidase; Beta-G1; Beta-glucuronidase; BG; BGLR; BGLR_HUMAN; Glucuronidase beta; Gur; Gus; Gus-r; Gus-s; Gus-t; Gus-u; GUSB; Gut; MPS7; Ac2-223.    

说 明 书    0.1ml  0.2ml      

研究领域    细胞生物  免疫学  信号转导      

抗体来源    Rabbit    

克隆类型    Polyclonal    

交叉反应    Human, Mouse, Rat, Chicken, Dog, Pig, Cow,     

产品应用    WB=1:100-500 ELISA=1:500-1000 IP=1:20-100 IHC-P=1:100-500 IHC-F=1:100-500 IF=1:100-500 β葡萄糖醛酸苷酶抗体(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.    

分 子 量    69kDa    

细胞定位    细胞浆     

性    状    Lyophilized or Liquid    

浓    度    1mg/1ml    

免 疫 原    KLH conjugated synthetic peptide derived from human GUSB/beta glucuronidase    

亚    型    IgG    

纯化方法    affinity purified by Protein A    

储 存 液    Preservative: 15mM Sodium Azide, Constituents: 1% BSA, 0.01M PBS, pH 7.4    

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.    

β葡萄糖醛酸苷酶抗体产品介绍    background:

Defects in GUSB are the cause of mucopolysaccharidosis type 7 (MPS7) ; also known as Sly syndrome. MPS7 is an autosomal recessive lysosomal storage disease characterized by inability to degrade glucuronic acid-containing glycosaminoglycans. The phenotype is highly variable, ranging from severe lethal hydrops fetalis to mild forms with survival into adulthood. Most patients with the intermediate phenotype show hepatomegaly, skeletal anomalies, coarse facies, and variable degrees of mental impairment. Mucopolysaccharidosis type 7 is associated with non-immune hydrops fetalis, a generalized edema of the fetus with fluid accumulation in the body cavities due to non-immune causes. Non-immune hydrops fetalis is not a diagnosis in itself but a symptom, a feature of many genetic disorders, and the end-stage of a wide variety of disorders.

Function:
Plays an important role in the degradation of dermatan and keratan sulfates.    

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