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, ELISAmore details
  • 種交差性
    交差種: Human
    交差が予測される動物種: Mouse, Rat, Rabbit, Horse, Chicken, Guinea pig, Cow, Cat, Dog, Pig
  • 免疫原

    Synthetic peptide corresponding to N terminal amino acids 144-193 (NELLMAISDS DYYTDDINRA CLFVPSIDVL NQNTLRIKET AQAMAQLSRW) of Human Ext2 (NP_000392).

  • ポジティブ・コントロール
    • 721_B cell lysate.

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 repeated freeze / thaw cycles.
  • バッファー
    Preservative: None
    Constituents: 2% Sucrose, PBS
  • Concentration information loading...
  • 精製度
    Immunogen affinity purified
  • 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 ab84582 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 Use a concentration of 1 µg/ml. Predicted molecular weight: 82 kDa. Good results were obtained when blocked with 5% non-fat dry milk in 0.05% PBS-T.
ELISA Use at an assay dependent concentration.

ELISA titre using peptide based assay: 1:312500.

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 (ab84582) at 1 µg/ml (in 5% skim milk / PBS buffer) + 721_B cell lysate at 10 µg

    Secondary
    HRP conjugated anti-Rabbit IgG at 1/50000 dilution

    Predicted band size : 82 kDa
    Observed band size : 95 kDa (why is the actual band size different from the predicted?)
    Additional bands at : 36 kDa. We are unsure as to the identity of these extra bands.

プロトコール

参考文献

ab84582 has not yet been referenced specifically in any publications.

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