Description

  • Product name

    Recombinant Human beta glucuronidase (GUSB) protein
    See all beta glucuronidase (GUSB) proteins and peptides
  • Expression system

    Wheat germ
  • Accession

  • Protein length

    Full length protein
  • Animal free

    No
  • Nature

    Recombinant
    • Species

      Human
    • Sequence

      GLQGGMLYPQESPSRECKELDGLWSFRADFSDNRRRGFEEQWYRRPLWES GPTVDMPVPSSFNDISQDWRLRHFVGWVWYEREVILPERWTQDLRTRVVL RIGSAHSYAIVWVNGVDTLEHEGGYLPFEADISNLVQVGPLPSRLRITIA INNTLTPTTLPPGTIQYLTDTSKYPKGYFVQNTYFDFFNYAGLQRSVLLY TTPTTYIDDITVTTSVEQDSGLVNYQISVKGSNLFKLEVRLLDAENKVVA NGTGTQGQLKVPGVSLWWPYLMHERPAYLYSLEVQLTAQTSLGPVSDFYT LPVGIRTVAVTKSQFLINGKPFYFHGVNKHEDADIRGKGFDWPLLVKDFN LLRWLGANAFRTSHYPYAEEVMQMCDRYGIVVIDECPGVGLALPQFFNNV SLHHHMQVMEEVVRRDKNHPAVVMWSVANEPASHLESAGYYLKMVIAHTK SLDPSRPVTFVSNSNYAADKGAPYVDVICLNSYYSWYHDYGHLELIQLQL ATQFENWYKKYQKPIIQSEYGAETIAGFHQDPPLMFTEEYQKSLLEQYHL GLDQKRRKYVVGELIWNFADFMTEQSPTRVLGNKKGIFTRQRQPKSAAFL LRERYWKIANETRYPHSVAKSQCLENSLFT
    • Predicted molecular weight

      95 kDa including tags
    • Amino acids

      22 to 651

Associated products

Specifications

Our Abpromise guarantee covers the use of ab114652 in the following tested applications.

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

  • Applications

    ELISA

    SDS-PAGE

    Western blot

  • Form

    Liquid
  • Additional notes

    H00002990-P01
  • Concentration information loading...

Preparation and Storage

  • Stability and Storage

    Shipped on dry ice. Upon delivery aliquot and store at -80ºC. Avoid freeze / thaw cycles.

    pH: 8.00
    Constituents: 0.3% Glutathione, 0.79% Tris HCl

General Info

  • Alternative names

    • Ac2-223
    • asd
    • Beta G1
    • Beta glucuronidase
    • Beta-G1
    • Beta-glucuronidase
    • BG
    • BGLR
    • BGLR_HUMAN
    • FLJ39445
    • Glucuronidase beta
    • Gur
    • Gus
    • Gus-r
    • Gus-s
    • Gus-t
    • Gus-u
    • GUSB
    • Gut
    • MPS7
    see all
  • 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

Images

  • 12.5% SDS-PAGE showing ab114652 at approximately 95.41kDa stained with Coomassie Blue.

References

ab114652 has not yet been referenced specifically in any publications.

Customer reviews and Q&As

There are currently no Customer reviews or Questions for ab114652.
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