Bioactive grade

Recombinant human beta glucuronidase (GUSB) protein (Active) (ab219458)

Description

  • Product name

    Recombinant human beta glucuronidase (GUSB) protein (Active)
    See all beta glucuronidase (GUSB) proteins and peptides
  • Biological activity

    Specific activity is > 1600 pmol/min/ug and is defined as the amount of enzyme that hydrolyze 1.0 pmole of 4-Methylumbelliferone to 4-Methylum-belliferyl-beta-D-glucosiduronic acid per minute at 37 degrees and pH 6.0

  • Purity

    > 90 % SDS-PAGE.
    purified by using conventional chromatography techniques.
  • Endotoxin level

    < 1.000 Eu/µg
  • Expression system

    Baculovirus infected insect cells
  • Accession

  • Protein length

    Full length protein
  • Animal free

    No
  • Nature

    Recombinant
    • Species

      Human
    • Sequence

      LQGGMLYPQESPSRECKELDGLWSFRADFSDNRRRGFEEQWYRRPLWESG PTVDMPVPSSFNDISQDWRLRHFVGWVWYEREVILPERWTQDLRTRVVLR IGSAHSYAIVWVNGVDTLEHEGGYLPFEADISNLVQVGPLPSRLRITIAI NNTLTPTTLPPGTIQYLTDTSKYPKGYFVQNTYFDFFNYAGLQRSVLLYT TPTTYIDDITVTTSVEQDSGLVNYQISVKGSNLFKLEVRLLDAENKVVAN GTGTQGQLKVPGVSLWWPYLMHERPAYLYSLEVQLTAQTSLGPVSDFYTL PVGIRTVAVTKSQFLINGKPFYFHGVNKHEDADIRGKGFDWPLLVKDFNL LRWLGANAFRTSHYPYAEEVMQMCDRYGIVVIDECPGVGLALPQFFNNVS LHHHMQVMEEVVRRDKNHPAVVMWSVANEPASHLESAGYYLKMVIAHTKS LDPSRPVTFVSNSNYAADKGAPYVDVICLNSYYSWYHDYGHLELIQLQLA TQFENWYKKYQKPIIQSEYGAETIAGFHQDPPLMFTEEYQKSLLEQYHLG LDQKRRKYVVGELIWNFADFMTEQSPTRVLGNKKGIFTRQRQPKSAAFLL RERYWKIANETRYPHSVAKSQCLENSLFTHHHHHH
    • Predicted molecular weight

      73 kDa including tags
    • Amino acids

      23 to 651
    • Tags

      His tag C-Terminus
    • Additional sequence information

      Mature protein without signal peptide.

Specifications

Our Abpromise guarantee covers the use of ab219458 in the following tested applications.

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

  • Applications

    SDS-PAGE

    Functional Studies

  • Form

    Liquid
  • Concentration information loading...

Preparation and Storage

  • Stability and Storage

    Shipped at 4°C. Store at +4°C short term (1-2 weeks). Upon delivery aliquot. Store at -20°C or -80°C. Avoid freeze / thaw cycle.

    pH: 7.4
    Constituents: 10% Glycerol, 90% PBS

    This product is an active protein and may elicit a biological response in vivo, handle with caution.

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

  • 15% SDS-PAGE using 3ug of ab219458.

References

ab219458 has not yet been referenced specifically in any publications.

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