Overview

  • Product name

    Anti-ARSB antibody [EPR9409]
    See all ARSB primary antibodies
  • Description

    Rabbit monoclonal [EPR9409] to ARSB
  • Host species

    Rabbit
  • Tested applications

    Suitable for: WB, IHC-P, Flow Cytmore details
    Unsuitable for: ICC or IP
  • Species reactivity

    Reacts with: Human
  • Immunogen

    Synthetic peptide within Human ARSB aa 450-550. The exact sequence is proprietary.

  • Positive control

    • Human placenta, HepG2, Human fetal muscle and Human fetal liver lysates; Human kidney and liver tissues
  • General notes

    Mouse, Rat: We have preliminary internal testing data to indicate this antibody may not react with these species. Please contact us for more information.

     

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

    This product is a recombinant rabbit monoclonal antibody.

Properties

Applications

Our Abpromise guarantee covers the use of ab150374 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 1/1000 - 1/10000. Predicted molecular weight: 46, 60 kDa.
IHC-P 1/50 - 1/100. Perform heat mediated antigen retrieval before commencing with IHC staining protocol.
Flow Cyt 1/10 - 1/100.

ab172730 - Rabbit monoclonal IgG, is suitable for use as an isotype control with this antibody.

 

  • Application notes
    Is unsuitable for ICC or IP.
  • 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 [EPR9409] (ab150374) at 1/1000 dilution

      Lane 1 : Human placenta lysates
      Lane 2 : HepG2 lysates
      Lane 3 : Human fetal muscle lysates
      Lane 4 : Human fetal liver lysates

      Lysates/proteins at 10 µg per lane.

      Secondary
      All lanes : HRP labelled goat anti-rabbit at 1/2000 dilution

      Predicted band size: 46, 60 kDa

    • Immunohistochemical analysis of paraffin embedded Human kidney tissue labeling ARSB with ab150374 at a 1/50 dilution.
    • Immunohistochemical analysis of paraffin embedded Human liver tissue labeling ARSB with ab150374 at a 1/50 dilution.

    References

    This product has been referenced in:

    • Uttarilli A  et al. Functional characterization of arylsulfatase B mutations in Indian patients with Maroteaux-Lamy syndrome (mucopolysaccharidosis type VI). Gene 599:19-27 (2017). WB . Read more (PubMed: 27826022) »
    See 1 Publication for this product

    Customer reviews and Q&As

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    Please note: All products are "FOR RESEARCH USE ONLY. NOT FOR USE IN DIAGNOSTIC PROCEDURES"
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