Overview

  • Product name

  • Description

    Rabbit polyclonal to ARSB
  • Host species

    Rabbit
  • Tested applications

    Suitable for: WBmore details
  • Species reactivity

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

    Recombinant fragment within Human ARSB (internal sequence). The exact sequence is proprietary.
    Database link: P15848

  • Positive control

    • WB: Mouse placenta tissue extract; rat testis tissue extract; HeLa and HepG2 whole cell extracts; HEK-293T transfected with ARSB, whole cell lysate.

Properties

Applications

Our Abpromise guarantee covers the use of ab228018 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: 60 kDa.

Target

  • Involvement in disease

    Defects in ARSB are the cause of mucopolysaccharidosis type 6 (MPS6) [MIM:253200]; also known as Maroteaux-Lamy syndrome. MPS6 is an autosomal recessive lysosomal storage disease characterized by intracellular accumulation of dermatan sulfate. Clinical features can include abnormal growth, short stature, stiff joints, skeletal malformations, corneal clouding, hepatosplenomegaly, and cardiac abnormalities. A wide variation in clinical severity is observed.
    Arylsulfatase B activity is defective in multiple sulfatase deficiency (MSD) [MIM:272200]. MSD is a disorder characterized by decreased activity of all known sulfatases. MSD is due to defects in SUMF1 resulting in the lack of post-translational modification of a highly conserved cysteine into 3-oxoalanine. It combines features of individual sulfatase deficiencies such as metachromatic leukodystrophy, mucopolysaccharidosis, chondrodysplasia punctata, hydrocephalus, ichthyosis, neurologic deterioration and developmental delay.
  • 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. This post-translational modification is severely defective in multiple sulfatase deficiency (MSD).
  • Cellular localization

    Lysosome.
  • Information by UniProt
  • Database links

  • Alternative names

    • Arsb antibody
    • ARSB_HUMAN antibody
    • Arylsulfatase B antibody
    • ArylsulfataseB antibody
    • ASB antibody
    • G4S antibody
    • MPS6 antibody
    • N acetylgalactosamine 4 sulfatase antibody
    • N-acetylgalactosamine-4-sulfatase antibody
    see all

Images

  • All lanes : Anti-ARSB antibody (ab228018) at 1/500 dilution

    Lane 1 : Non-transfected HEK-293T (human epithelial cell line from embryonic kidney transformed with large T antigen) whole cell lysate.
    Lane 2 : HEK-293T transfected with ARSB, whole cell lysate

    Developed using the ECL technique.

    Predicted band size: 60 kDa



    12% SDS-PAGE

  • Anti-ARSB antibody (ab228018) at 1/500 dilution + Mouse placenta tissue extract at 50 µg

    Secondary
    HRP-conjugated anti-rabbit IgG antibody

    Developed using the ECL technique.

    Predicted band size: 60 kDa



    10% SDS-PAGE

  • Anti-ARSB antibody (ab228018) at 1/500 dilution + Rat testis tissue extract at 50 µg

    Secondary
    HRP-conjugated anti-rabbit IgG antibody

    Developed using the ECL technique.

    Predicted band size: 60 kDa



    10% SDS-PAGE

  • All lanes : Anti-ARSB antibody (ab228018) at 1/1000 dilution

    Lane 1 : HeLa (human epithelial cell line from cervix adenocarcinoma) whole cell extract
    Lane 2 : HepG2 (human liver hepatocellular carcinoma cell line) whole cell extract

    Lysates/proteins at 30 µg per lane.

    Developed using the ECL technique.

    Predicted band size: 60 kDa



    10% SDS-PAGE

References

ab228018 has not yet been referenced specifically in any publications.

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