製品の概要

  • 製品名
  • 製品の詳細
    Goat polyclonal to EVC2
  • アプリケーション
    適用あり: ELISA, IHC-Pmore details
  • 種交差性
    交差種: Human
  • 免疫原

    Synthetic peptide:

    C-LNAKKAMRALGMD

    , corresponding to C terminal amino acids 1296-1308 of Human EVC2.

  • ポジティブ・コントロール
    • Human placenta and testis tissues

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
    pH: 7.30
    Preservative: 0.02% Sodium azide
    Constituents: 99% Tris buffered saline, 0.5% BSA
  • 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 ab113924 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
ELISA 1/16000.
IHC-P Use a concentration of 3.75 µg/ml.

ターゲット情報

  • 関連性
    EVC2 is an integral membrane protein that plays a vital role in bone formation and skeletal development. Defects in EVC2 are a cause of Ellis-van Creveld syndrome (EVC), also known as chondroectodermal dysplasia. EVC is an autosomal recessive disorder characterized by the clinical tetrad of chondrodystrophy, polydactyly, ectodermal dysplasia and cardiac anomalies. Patients manifest short-limb dwarfism, short ribs, postaxial polydactyly and dysplastic nails and teeth. Congenital heart defects, most commonly an atrioventricular septal defect, are observed in 60% of affected individuals. Defects in Defects in EVC2 are also a cause of acrofacial dysostosis Weyers type (WAD), also known as Curry-Hall syndrome. Acrofacial dysostoses are a heterogeneous group of disorders combining limb defects with facial abnormalities. WAD is an autosomal dominant disorder characterized by dysplastic nails, postaxial polydactyly, acrofacial dysostosis, short limbs and short stature. The phenotype is milder than Ellis-van Creveld syndrome.
  • 細胞内局在
    Membrane; Multi-pass membrane protein
  • 参照データベース
  • 別名
    • Ellis van Creveld syndrome 2 antibody
    • LBN antibody
    • Limbin antibody

画像

  • ab113924, at 3.75 µg/ml, staining EVC2 in formalin fixed, paraffin embedded Human placenta tissue by Immunohistochemistry.
  • ab113924, at 3.75 µg/ml, staining EVC2 in formalin fixed, paraffin embedded Human testis tissue by Immunohistochemistry.

プロトコール

参考文献

ab113924 has not yet been referenced specifically in any publications.

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Please note: All products are "FOR RESEARCH USE ONLY AND ARE NOT INTENDED FOR DIAGNOSTIC OR THERAPEUTIC USE"

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