Anti-beta glucuronidase (GUSB)抗体(ab88567)

概述

  • 产品名称Anti-beta glucuronidase (GUSB)抗体
    参阅全部 beta glucuronidase (GUSB) 一抗
  • 描述
    小鼠多克隆抗体to beta glucuronidase (GUSB)
  • 经测试应用适用于: WBmore details
  • 种属反应性
    与反应: Human
  • 免疫原

    Full length Human beta glucuronidase (GUSB) protein (NP_000172.1)

  • 阳性对照
    • Human liver tissue lysate and beta glucuronidase (GUSB) transfected 293T cell lysate.

性能

应用

Our Abpromise guarantee covers the use of ab88567 in the following tested applications.

The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.

应用 Ab评论 说明
WB Use a concentration of 1 µg/ml. Predicted molecular weight: 75 kDa.

靶标

  • 功能Plays an important role in the degradation of dermatan and keratan sulfates.
  • 疾病相关Defects in GUSB are the cause of mucopolysaccharidosis type 7 (MPS7) [MIM:253220]; 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.
    Note=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.
  • 序列相似性Belongs to the glycosyl hydrolase 2 family.
  • 翻译后修饰N-linked glycosylated with 3 to 4 oligosaccharide chains.
  • 细胞定位Lysosome.
  • Information by UniProt
  • 数据库链接
  • 别名
    • Ac2-223 antibody
    • asd antibody
    • Beta G1 antibody
    • Beta glucuronidase antibody
    • Beta-G1 antibody
    • Beta-glucuronidase antibody
    • BG antibody
    • BGLR antibody
    • BGLR_HUMAN antibody
    • FLJ39445 antibody
    • Glucuronidase beta antibody
    • Gur antibody
    • Gus antibody
    • Gus-r antibody
    • Gus-s antibody
    • Gus-t antibody
    • Gus-u antibody
    • GUSB antibody
    • Gut antibody
    • MPS7 antibody
    see all

Anti-beta glucuronidase (GUSB) antibody 图像

  • Anti-beta glucuronidase (GUSB) antibody (ab88567) at 1 µg/ml + human liver tissue lysate at 50 µg

    Predicted band size : 75 kDa
    Observed band size : 60 kDa (why is the actual band size different from the predicted?)
  • All lanes : Anti-beta glucuronidase (GUSB) antibody (ab88567) at 1 µg/ml

    Lane 1 : beta glucuronidase (GUSB) transfected 293T cell lysate
    Lane 2 : Non transfected 293T cell lysate

    Lysates/proteins at 25 µg per lane.


    Predicted band size : 75 kDa
    Observed band size : 85 kDa (why is the actual band size different from the predicted?)

Anti-beta glucuronidase (GUSB) antibody (ab88567)参考文献

ab88567 has not yet been referenced specifically in any publications.

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