Anti-beta glucuronidase (GUSB) antibody (ab88567)

Overview

  • Product nameAnti-beta glucuronidase (GUSB) antibody
    See all beta glucuronidase (GUSB) primary antibodies
  • Description
    Mouse polyclonal to beta glucuronidase (GUSB)
  • Tested applicationsSuitable for: WBmore details
  • Species reactivity
    Reacts with: Human
  • Immunogen

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

  • Positive control
    • Human liver tissue lysate and beta glucuronidase (GUSB) transfected 293T cell lysate.

Properties

Applications

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.

Application Abreviews Notes
WB Use a concentration of 1 µg/ml. Predicted molecular weight: 75 kDa.

Target

  • FunctionPlays an important role in the degradation of dermatan and keratan sulfates.
  • Involvement in diseaseDefects 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.
  • Sequence similaritiesBelongs to the glycosyl hydrolase 2 family.
  • Post-translational
    modifications
    N-linked glycosylated with 3 to 4 oligosaccharide chains.
  • Cellular localizationLysosome.
  • Information by UniProt
  • Database links
  • Alternative names
    • 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 images

  • 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?)

References for Anti-beta glucuronidase (GUSB) antibody (ab88567)

ab88567 has not yet been referenced specifically in any publications.

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