Recombinant
RabMAb

Recombinant Anti-SGSH/HSS antibody [EPR17312] (ab200346)

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Overview

  • Product name

    Anti-SGSH/HSS antibody [EPR17312]
    See all SGSH/HSS primary antibodies

  • Description

    Rabbit monoclonal [EPR17312] to SGSH/HSS

  • Host species

    Rabbit

  • Tested applications

    Suitable for: ICC/IF, WBmore details

  • Species reactivity

    Reacts with: Human

  • Immunogen

    Recombinant fragment within Human SGSH/HSS aa 250 to the C-terminus. The exact sequence is proprietary.
    Database link: P51688

  • Positive control

    • WB: HepG2 and MCF7 whole cell lysates; Human prostate cancer lysate; ICC/IF: MCF7 and HepG2 cells.

  • General notes

     

     

     This product was previously labelled as SGSH

     

    This product is a recombinant monoclonal antibody, which offers several advantages including:

    • - High batch-to-batch consistency and reproducibility
    • - Improved sensitivity and specificity
    • - Long-term security of supply
    • - Animal-free production
    For more information see here.

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

Properties

Applications

Our Abpromise guarantee covers the use of ab200346 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
ICC/IF 1/250.
WB 1/1000. Detects a band of approximately 57 kDa (predicted molecular weight: 57 kDa).

Target

  • Involvement in disease

    Defects in SGSH are the cause of mucopolysaccharidosis type 3A (MPS3A) [MIM:252900]; also known as Sanfilippo syndrome A. MPS3A is a severe 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. MPS3A is characterized by earlier onset, rapid progression of symptoms and shorter survival.

  • Sequence similarities

    Belongs to the sulfatase family.

  • Post-translational
    modifications

    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

      • Heparan sulfate sulfatase antibody
      • Heparan sulphate sulphatase antibody
      • HSS antibody
      • MPS 3A antibody
      • MPS3 A antibody
      • MPS3A antibody
      • Mucopolysaccharidosis type IIIA antibody
      • N sulfoglucosamine sulfohydrolase (sulfamidase) antibody
      • N sulfoglucosamine sulfohydrolase antibody
      • N-sulphoglucosamine sulphohydrolase antibody
      • SFMD antibody
      • SGSH antibody
      • SPHM_HUMAN antibody
      • Sulfoglucosamine sulfamidase antibody
      • Sulphamidase antibody
      • Sulphoglucosamine sulphamidase antibody
      see all

    Images

    • Western blot - Anti-SGSH/HSS antibody [EPR17312] (ab200346)
      Western blot - Anti-SGSH/HSS antibody [EPR17312] (ab200346)
      Anti-SGSH/HSS antibody [EPR17312] (ab200346) at 1/1000 dilution + HepG2 (Human liver hepatocellular carcinoma) whole cell lysate at 20 µg

      Secondary
      Goat Anti-Rabbit IgG, (H+L), Peroxidase conjugated at 1/1000 dilution

      Predicted band size: 57 kDa
      Observed band size: 57 kDa


      Exposure time: 3 minutes


      Blocking/Dilution buffer: 5% NFDM/TBST.

    • Western blot - Anti-SGSH/HSS antibody [EPR17312] (ab200346)
      Western blot - Anti-SGSH/HSS antibody [EPR17312] (ab200346)
      Anti-SGSH/HSS antibody [EPR17312] (ab200346) at 1/10000 dilution + MCF7 (Human breast adenocarcinoma cell line) whole cell lysate at 10 µg

      Secondary
      Goat Anti-Rabbit IgG, (H+L), Peroxidase conjugated at 1/1000 dilution

      Predicted band size: 57 kDa
      Observed band size: 57 kDa


      Exposure time: 3 minutes


      Blocking/Dilution buffer: 5% NFDM/TBST.

    • Western blot - Anti-SGSH/HSS antibody [EPR17312] (ab200346)
      Western blot - Anti-SGSH/HSS antibody [EPR17312] (ab200346)
      Anti-SGSH/HSS antibody [EPR17312] (ab200346) at 1/10000 dilution + Human prostate cancer lysate at 10 µg

      Secondary
      Anti-Rabbit IgG (HRP), specific to the non-reduced form of IgG at 1/1000 dilution

      Predicted band size: 57 kDa
      Observed band size: 57 kDa


      Exposure time: 30 seconds


      Blocking/Dilution buffer: 5% NFDM/TBST.

    • Immunocytochemistry/ Immunofluorescence - Anti-SGSH/HSS antibody [EPR17312] (ab200346)
      Immunocytochemistry/ Immunofluorescence - Anti-SGSH/HSS antibody [EPR17312] (ab200346)

      Immunofluorescent analysis of 100% methanol-fixed, 0.1% Triton X-100 permeabilized MCF7 (Human breast adenocarcinoma cell line) cells labeling SGSH/HSS with ab200346 at 1/250 dilution, followed by Goat anti-rabbit IgG (Alexa Fluor® 488) (ab150077) secondary antibody at 1/500 dilution (green). Cytoplasm staining on MCF7 cell line was observed. The nuclear counter stain is DAPI (blue). Tubulin is detected with ab7291 (anti-Tubulin mouse mAb) at 1/1000 dilution and ab150120 (AlexaFluor®594 Goat anti-Mouse secondary) at 1/500 dilution (red).

      The negative controls are as follows:
      -ve control 1: ab200346 at 1/250 dilution followed by ab150120 (AlexaFluor®594 Goat anti-Mouse secondary) at 1/500 dilution.
      -ve control 2: ab7291 (anti-Tubulin mouse mAb) at 1/1000 dilution followed by ab150077 (Alexa Fluor®488 Goat Anti-Rabbit IgG H&L) at 1/500 dilution.

    • Immunocytochemistry/ Immunofluorescence - Anti-SGSH/HSS antibody [EPR17312] (ab200346)
      Immunocytochemistry/ Immunofluorescence - Anti-SGSH/HSS antibody [EPR17312] (ab200346)

      Immunofluorescent analysis of 100% methanol-fixed, 0.1% Triton X-100 permeabilized HepG2 (Human liver hepatocellular carcinoma) cells labeling SGSH/HSS with ab200346 at 1/250 dilution, followed by Goat anti-rabbit IgG (Alexa Fluor® 488) (ab150077) secondary antibody at 1/500 dilution (green). Cytoplasm staining on HepG2 cell line was observed. The nuclear counter stain is DAPI (blue). Tubulin is detected with ab7291 (anti-Tubulin mouse mAb) at 1/1000 dilution and ab150120 (AlexaFluor®594 Goat anti-Mouse secondary) at 1/500 dilution (red).

      The negative controls are as follows:
      -ve control 1: ab200346 at 1/250 dilution followed by ab150120 (AlexaFluor®594 Goat anti-Mouse secondary) at 1/500 dilution.
      -ve control 2: ab7291 (anti-Tubulin mouse mAb) at 1/1000 dilution followed by ab150077 (Alexa Fluor®488 Goat Anti-Rabbit IgG H&L) at 1/500 dilution.

    References

    This product has been referenced in:

    • Knottnerus SJG  et al. Prediction of phenotypic severity in mucopolysaccharidosis type IIIA. Ann Neurol 82:686-696 (2017). WB, ICC/IF ; Human . Read more (PubMed: 29023963) »
    See 1 Publication for this product

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