Overview

  • Product nameAnti-GNS antibody
    See all GNS primary antibodies
  • Description
    Mouse polyclonal to GNS
  • Tested applicationsSuitable for: WBmore details
  • Species reactivity
    Reacts with: Human
  • Immunogen

    Full length human GNS (NP_002067.1).

  • Positive control
    • GNS transfected 293T lysate.

Properties

Applications

Our Abpromise guarantee covers the use of ab69971 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 1/500 - 1/1000. Detects a band of approximately 75 kDa (predicted molecular weight: 63 kDa).

Target

  • Involvement in diseaseDefects 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 similaritiesBelongs 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 localizationLysosome.
  • 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

Anti-GNS antibody images

  • All lanes : Anti-GNS antibody (ab69971) at 1/500 dilution

    Lane 1 : GNS transfected 293T lysate
    Lane 2 : Non transfected 293T lysate

    Lysates/proteins at 25 µg per lane.

    Secondary
    Goat Anti-Mouse IgG (H&L)-HRP at 1/2500 dilution

    Predicted band size : 63 kDa
    Observed band size : 75 kDa (why is the actual band size different from the predicted?)

References for Anti-GNS antibody (ab69971)

ab69971 has not yet been referenced specifically in any publications.

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