Overview

  • Product name

  • Description

    Rabbit polyclonal to Ext2
  • Host species

    Rabbit
  • Tested applications

    Suitable for: WB, IHC-Pmore details
  • Species reactivity

    Reacts with: Human
  • Immunogen

    Synthetic peptide conjugated to KLH, corresponding to a region within internal sequence amino acids 188-218 of Human Ext2

  • Positive control

    • MDA-MB468 cell lysate. Human brain tissue.

Properties

  • Form

    Liquid
  • Storage instructions

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

    Preservative: 0.09% Sodium azide
    Constituent: PBS
  • Concentration information loading...
  • Purity

    Immunogen affinity purified
  • Purification notes

    Purified through a protein A column, followed by peptide affinity purification.
  • Clonality

    Polyclonal
  • Isotype

    IgG
  • Research areas

Applications

Our Abpromise guarantee covers the use of ab102843 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 1/100 - 1/500. Predicted molecular weight: 82 kDa.
IHC-P 1/50 - 1/100. Perform heat mediated antigen retrieval with citrate buffer pH 6 before commencing with IHC staining protocol.

Target

  • Function

    Glycosyltransferase required for the biosynthesis of heparan-sulfate. The EXT1/EXT2 complex possesses substantially higher glycosyltransferase activity than EXT1 or EXT2 alone. Appears to be a tumor suppressor.
  • Tissue specificity

    Ubiquitous.
  • Pathway

    Protein modification; protein glycosylation.
  • Involvement in disease

    Defects in EXT2 are a cause of hereditary multiple exostoses type 2 (EXT2) [MIM:133701]. EXT is a genetically heterogeneous bone disorder caused by genes segregating on human chromosomes 8, 11, and 19 and designated EXT1, EXT2 and EXT3 respectively. EXT is a dominantly inherited skeletal disorder primarily affecting endochondral bone during growth. The disease is characterized by formation of numerous cartilage-capped, benign bone tumors (osteocartilaginous exostoses or osteochondromas) that are often accompanied by skeletal deformities and short stature. In a small percentage of cases exostoses have exhibited malignant transformation resulting in an osteosarcoma or chondrosarcoma. Osteochondromas development can also occur as a sporadic event.
    Defects in EXT2 are the cause of Potocki-Shaffer syndrome (PSS) [MIM:601224]. It is a contiguous gene syndrome due to proximal deletion of chromosome 11p11.2, including EXT2 and ALX4.
  • Sequence similarities

    Belongs to the glycosyltransferase 47 family.
  • Cellular localization

    Endoplasmic reticulum membrane. Golgi apparatus membrane. The EXT1/EXT2 complex is localized in the Golgi apparatus.
  • Information by UniProt
  • Database links

  • Alternative names

    • Exostoses (multiple) 2 antibody
    • Exostosin 2 antibody
    • Exostosin-2 antibody
    • EXT2 antibody
    • EXT2_HUMAN antibody
    • Glucuronosyl N acetylglucosaminyl proteoglycan 4 alpha N acetylglucosaminyltransferase antibody
    • Glucuronosyl-N-acetylglucosaminyl-proteoglycan/N-acetylglucosaminyl-proteoglycan 4-alpha-N-acetylglucosaminyltransferase antibody
    • Multiple exostoses protein 2 antibody
    • N acetylglucosaminyl proteoglycan 4 beta glucuronosyltransferase antibody
    • Putative tumor suppressor protein EXT2 antibody
    • SOTV antibody
    see all

Images

  • Anti-Ext2 antibody (ab102843) at 2 µg/ml + MDA-MB468 cell lysate at 35 µg

    Predicted band size: 82 kDa

  • ab102843, at 1/50 dilution, staining EXT2 in formalin-fixed and paraffin-embedded human brain by immunohistochemistry.

References

This product has been referenced in:

  • Tian C  et al. A splice mutation and mRNA decay of EXT2 provoke hereditary multiple exostoses. PLoS One 9:e94848 (2014). WB ; Human . Read more (PubMed: 24728384) »
See 1 Publication for this product

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