Description

  • Product name

    Recombinant Human GNS protein
  • Purity

    > 95 % SDS-PAGE.
    ab151650 was determined to be >95% pure by SEC-HPLC and reducing SDS-PAGE.
  • Endotoxin level

    < 1.000 Eu/µg
  • Expression system

    HEK 293 cells
  • Accession

  • Protein length

    Full length protein
  • Animal free

    No
  • Nature

    Recombinant
    • Species

      Human
    • Sequence

      VFGVAAGTRRPNVVLLLTDDQDEVLGGMTPLKKTKALIGEMGMTFSSAYV PSALCCPSRASILTGKYPHNHHVVNNTLEGNCSSKSWQKIQEPNTFPAIL RSMCGYQTFFAGKYLNEYGAPDAGGLEHVPLGWSYWYALEKNSKYYNYTL SINGKARKHGENYSVDYLTDVLANVSLDFLDYKSNFEPFFMMIATPAPHS PWTAAPQYQKAFQNVFAPRNKNFNIHGTNKHWLIRQAKTPMTNSSIQFLD NAFRKRWQTLLSVDDLVEKLVKRLEFTGELNNTYIFYTSDNGYHTGQFSL PIDKRQLYEFDIKVPLLVRGPGIKPNQTSKMLVANIDLGPTILDIAGYDL NKTQMDGMSLLPILRGASNLTWRSDVLVEYQGEGRNVTDPTCPSLSPGVS QCFPDCVCEDAYNNTYACVRTMSALWNLQYCEFDDQEVFVEVYNLTADPD QITNIAKTIDPELLGKMNYRLMMLQSCSGPTCRTPGVFDPGYRFDPRLMF SNRGSVRTRRFSKHLLVDHHHHHH
    • Predicted molecular weight

      58 kDa
    • Amino acids

      37 to 552
    • Tags

      His tag C-Terminus

Specifications

Our Abpromise guarantee covers the use of ab151650 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

    HPLC

  • Form

    Liquid
  • Additional notes

    ab151650 was produced by a mammalian cell expression system in HEK293.
  • Concentration information loading...

Preparation and Storage

  • Stability and Storage

    Shipped at 4°C. Upon delivery aliquot and store at -20°C. Avoid repeated freeze / thaw cycles.

    pH: 8.00
    Constituents: 0.32% Tris HCl, 0.88% Sodium chloride

General Info

  • Alternative names

    • 2610016K11Rik
    • AU042285
    • C87209
    • G6S
    • Glucosamine (N-acetyl) 6 sulfatase
    • Glucosamine 6 sulfatase
    • Glucosamine-6-sulfatase
    • GNS
    • GNS_HUMAN
    • MGC21274
    • N acetylglucosamine 6 sulfatase [Precursor]
    • N-acetylglucosamine-6-sulfatase
    • N28088
    see all
  • Involvement in disease

    Defects in GNS are the cause of mucopolysaccharidosis type 3D (MPS3D) [MIM:252940]; also known as Sanfilippo D syndrome. MPS3D is a form of mucopolysaccharidosis type 3, an autosomal recessive lysosomal storage disease due to impaired degradation of heparan sulfate. MPS3 is characterized by severe central nervous system degeneration, but only mild somatic disease. Onset of clinical features usually occurs between 2 and 6 years; severe neurologic degeneration occurs in most patients between 6 and 10 years of age, and death occurs typically during the second or third decade of life.
  • Sequence similarities

    Belongs to the sulfatase family.
  • Post-translational
    modifications

    The form A (78 kDa) is processed by internal peptidase cleavage to a 32 kDa N-terminal species (form B) and a 48 kDa C-terminal species.
    The conversion to 3-oxoalanine (also known as C-formylglycine, FGly), of a serine or cysteine residue in prokaryotes and of a cysteine residue in eukaryotes, is critical for catalytic activity.
  • Cellular localization

    Lysosome.
  • Information by UniProt

References

ab151650 has not yet been referenced specifically in any publications.

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