Overview

  • Product name

  • Description

    Rabbit polyclonal to GNS
  • Host species

    Rabbit
  • Tested applications

    Suitable for: IHC-Pmore details
  • Species reactivity

    Reacts with: Human
    Predicted to work with: Mouse, Goat, Cow
  • Immunogen

    Recombinant fragment corresponding to Human GNS aa 293-552.
    Sequence:

    WQTLLSVDDLVEKLVKRLEFTGELNNTYIFYTSDNGYHTGQFSLPIDKRQ LYEFDIKVPLLVRGPGIKPNQTSKMLVANIDLGPTILDIAGYDLNKTQMD GMSLLPILRGASNLTWRSDVLVEYQGEGRNVTDPTCPSLSPGVSQCFPDC VCEDAYNNTYACVRTMSALWNLQYCEFDDQEVFVEVYNLTADPDQITNIA KTIDPELLGKMNYRLMMLQSCSGPTCRTPGVFDPGYRFDPRLMFSNRGSV RTRRFSKHLL


    Database link: P15586

  • Positive control

    • IHC-P: Human testis and kidney tissues.

Properties

Applications

Our Abpromise guarantee covers the use of ab230037 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
IHC-P 1/20 - 1/200.

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

  • Paraffin-embedded human testis tissue stained for GNS using ab230037 at 1/100 dilution in immunohistochemical analysis.

  • Paraffin-embedded human kidney tissue stained for GNS using ab230037 at 1/100 dilution in immunohistochemical analysis.

References

ab230037 has not yet been referenced specifically in any publications.

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