Overview

  • Product nameAnti-SGSH antibody
    See all SGSH primary antibodies
  • Description
    Rabbit polyclonal to SGSH
  • Tested applicationsSuitable for: WBmore details
  • Species reactivity
    Reacts with: Human
    Predicted to work with: Mouse, Dog
  • Immunogen

    Recombinant fragment, corresponding a region within amino acids 44-200 of Human SGSH

  • Positive control
    • 293T whole cell lysate

Properties

Associated products

Applications

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.

Target

  • Involvement in diseaseDefects 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 similaritiesBelongs 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 localizationLysosome.
  • 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

Anti-SGSH antibody images

  • Anti-SGSH antibody (ab96030) at 1/1000 dilution + 293T whole cell lysate at 30 µg

    Predicted band size : 57 kDa

References for Anti-SGSH antibody (ab96030)

ab96030 has not yet been referenced specifically in any publications.

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