製品の概要

  • 製品名
  • 製品の詳細
    Rabbit polyclonal to Ext2
  • アプリケーション
    適用あり: WB, IHC-Pmore details
  • 種交差性
    交差種: Human
  • 免疫原

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

  • ポジティブ・コントロール
    • MDA-MB468 cell lysate. Human brain tissue.

製品の特性

  • 製品の状態
    Liquid
  • 保存方法
    Shipped at 4°C. Upon delivery aliquot and store at -20°C. Avoid freeze / thaw cycles.
  • バッファー
    Preservative: 0.09% Sodium Azide
    Constituents: PBS
  • Concentration information loading...
  • 精製度
    Immunogen affinity purified
  • 特記事項(精製)
    Purified through a protein A column, followed by peptide affinity purification.
  • ポリ/モノ
    ポリクローナル
  • アイソタイプ
    IgG
  • 研究分野

アプリケーション

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.

アプリケーション Abreviews 特記事項
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.

ターゲット情報

  • 機能
    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.
  • 組織特異性
    Ubiquitous.
  • パスウェイ
    Protein modification; protein glycosylation.
  • 関連疾患
    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.
  • 配列類似性
    Belongs to the glycosyltransferase 47 family.
  • 細胞内局在
    Endoplasmic reticulum membrane. Golgi apparatus membrane. The EXT1/EXT2 complex is localized in the Golgi apparatus.
  • Information by UniProt
  • 参照データベース
  • 別名
    • 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

画像

  • 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.

参考文献

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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