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    sgshhss-antibody-ab96030.pdf

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Tags & Cell Markers Subcellular Markers Organelles Lysosome
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Anti-SGSH/HSS antibody (ab96030)

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Western blot - Anti-SGSH/HSS antibody (ab96030)

    Key features and details

    • Rabbit polyclonal to SGSH/HSS
    • Suitable for: WB
    • Reacts with: Human
    • Isotype: IgG

    You may also be interested in

    Protein
    Product image
    Recombinant Human SGSH/HSS protein (ab116779)
    Secondary
    Product image
    Goat Anti-Rabbit IgG H&L (HRP) (ab205718)

    View more associated products

    Overview

    • Product name

      Anti-SGSH/HSS antibody
      See all SGSH/HSS primary antibodies
    • Description

      Rabbit polyclonal to SGSH/HSS
    • Host species

      Rabbit
    • Tested applications

      Suitable for: WBmore details
    • Species reactivity

      Reacts with: Human
      Predicted to work with: Mouse, Dog
    • Immunogen

      Recombinant fragment corresponding to Human SGSH/HSS aa 44-200.

    • Positive control

      • 293T whole cell lysate
    • General notes

       This product was previously labelled as SGSH

       

      The Life Science industry has been in the grips of a reproducibility crisis for a number of years. Abcam is leading the way in addressing this with our range of recombinant monoclonal antibodies and knockout edited cell lines for gold-standard validation. Please check that this product meets your needs before purchasing.

      If you have any questions, special requirements or concerns, please send us an inquiry and/or contact our Support team ahead of purchase. Recommended alternatives for this product can be found below, along with publications, customer reviews and Q&As

    Properties

    • Form

      Liquid
    • Storage instructions

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

      pH: 7.00
      Preservative: 0.01% Thimerosal (merthiolate)
      Constituents: 59.99% PBS, 40% Glycerol (glycerin, glycerine)
    • Concentration information loading...
    • Purity

      Protein A purified
    • Clonality

      Polyclonal
    • Isotype

      IgG
    • Research areas

      • Tags & Cell Markers
      • Subcellular Markers
      • Organelles
      • Lysosome
      • Neuroscience
      • Neurology process
      • Neurodegenerative disease
      • Other
      • Signal Transduction
      • Cytoskeleton / ECM
      • HSPGs

    Associated products

    • Compatible Secondaries

      • Goat Anti-Rabbit IgG H&L (Alexa Fluor® 488) (ab150077)
      • Goat Anti-Rabbit IgG H&L (HRP) (ab205718)
    • Isotype control

      • Rabbit IgG, polyclonal - Isotype Control (ChIP Grade) (ab171870)
    • Positive Controls

      • HEK-293 whole cell lysate (ab7902)
    • Recombinant Protein

      • Recombinant Human SGSH/HSS protein (ab116779)

    Applications

    The Abpromise guarantee

    Our Abpromise guarantee covers the use of ab96030 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 - 1/3000. Predicted molecular weight: 57 kDa.
    Notes
    WB
    1/500 - 1/3000. 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.
    • Target information above from: UniProt accession P51688 The UniProt Consortium
      The Universal Protein Resource (UniProt) in 2010
      Nucleic Acids Res. 38:D142-D148 (2010) .

      Information by UniProt
    • Database links

      • Entrez Gene: 6448 Human
      • Entrez Gene: 27029 Mouse
      • Omim: 605270 Human
      • SwissProt: P51688 Human
      • Unigene: 31074 Human
      • Unigene: 103788 Mouse
      • 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 (ab96030)
        Western blot - Anti-SGSH/HSS antibody (ab96030)
        Anti-SGSH/HSS antibody (ab96030) at 1/1000 dilution + 293T whole cell lysate at 30 µg

        Predicted band size: 57 kDa



        7.5% SDS Page

      Protocols

      • Western blot protocols

      Click here to view the general protocols

      Datasheets and documents

      • SDS download

      • Datasheet download

        Download

      References (0)

      Publishing research using ab96030? Please let us know so that we can cite the reference in this datasheet.

      ab96030 has not yet been referenced specifically in any publications.

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