Overview

  • Product name

    Anti-GNS antibody [EPR8329(2)]
    See all GNS primary antibodies
  • Description

    Rabbit monoclonal [EPR8329(2)] to GNS
  • Host species

    Rabbit
  • Tested applications

    Suitable for: WB, IPmore details
    Unsuitable for: Flow Cyt,ICC/IF or IHC-P
  • Species reactivity

    Reacts with: Human
  • Immunogen

    Synthetic peptide, corresponding to residues in Human GNS (UniProt: P15586).

  • Positive control

    • PC3, NCI-H460 and 293T cell lysates.
  • General notes

    Mouse, Rat: We have preliminary internal testing data to indicate this antibody may not react with these species. Please contact us for more information.

     

    Our RabMAb® technology is a patented hybridoma-based technology for making rabbit monoclonal antibodies. For details on our patents, please refer to RabMab® patents.

    This product is a recombinant rabbit monoclonal antibody.

Properties

Applications

Our Abpromise guarantee covers the use of ab154177 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 1/10000 - 1/50000. Predicted molecular weight: 62 kDa.
IP 1/10 - 1/100.
  • Application notes
    Is unsuitable for Flow Cyt,ICC/IF or IHC-P.
  • Target

    • 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
    • Database links

    • Alternative names

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

    Images

    • All lanes : Anti-GNS antibody [EPR8329(2)] (ab154177) at 1/10000 dilution

      Lane 1 : PC3 cell lysate
      Lane 2 : NCI-H460 cell lysate
      Lane 3 : 293T cell lysate

      Lysates/proteins at 10 µg per lane.

      Secondary
      All lanes : Goat anti-rabbit HRP at 1/2000 dilution

      Predicted band size: 62 kDa

    References

    ab154177 has not yet been referenced specifically in any publications.

    Customer reviews and Q&As

    There are currently no Customer reviews or Questions for ab154177.
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