Overview

  • Product name

    Anti-beta glucuronidase (GUSB) antibody
    See all beta glucuronidase (GUSB) primary antibodies
  • Description

    Rabbit polyclonal to beta glucuronidase (GUSB)
  • Host species

    Rabbit
  • Tested applications

    Suitable for: WBmore details
  • Species reactivity

    Reacts with: Human
    Predicted to work with: Mouse, Rat
  • Immunogen

    Synthetic peptide derived from internal amino acids of Human beta glucuronidase (GUSB).

  • Positive control

    • COLO205 cell lysate.

Properties

Applications

Our Abpromise guarantee covers the use of ab136860 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 1/500 - 1/1000. Predicted molecular weight: 75 kDa.

Target

  • Function

    Plays an important role in the degradation of dermatan and keratan sulfates.
  • Involvement in disease

    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.
  • Sequence similarities

    Belongs to the glycosyl hydrolase 2 family.
  • Post-translational
    modifications

    N-linked glycosylated with 3 to 4 oligosaccharide chains.
  • Cellular localization

    Lysosome.
  • 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

Images

  • Anti-beta glucuronidase (GUSB) antibody (ab136860) at 1/500 dilution + COLO205 cell lysate at 30 µg

    Predicted band size: 75 kDa

References

ab136860 has not yet been referenced specifically in any publications.

Customer reviews and Q&As

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