製品の概要

  • 製品名Anti-GGCX antibody
    GGCX 一次抗体 製品一覧
  • 製品の詳細
    Goat polyclonal to GGCX
  • 特異性This antibody is expected to recognize both isoforms (NP_000812.2; NP_001135741.1).
  • アプリケーション適用あり: WBmore details
  • 種交差性
    交差種: Human
    交差が予測される動物種: Dog, Orangutan
  • 免疫原

    Synthetic peptide:

    C-PPESNPDPVHSE

    , corresponding to C terminal amino acids 746-757 of Human GGCX isoform 1 (NP_000812.2) or amino acids 689-700 of isoform 2 (NP_001135741.1).

  • ポジティブ・コントロール
    • Human Muscle lysates.

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: 0.02% Sodium Azide
    Constituents: 0.5% BSA, Tris buffered saline, pH 7.3
  • Concentration information loading...
  • 精製度Immunogen affinity purified
  • 特記事項(精製)Purified from goat serum by ammonium sulphate precipitation followed by antigen affinity chromatography using the immunizing peptide.
  • 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 ab106924 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 0.3 - 1 µg/ml. Predicted molecular weight: 88 kDa.

ターゲット情報

  • 機能Mediates the vitamin K-dependent carboxylation of glutamate residues to calcium-binding gamma-carboxyglutamate (Gla) residues with the concomitant conversion of the reduced hydroquinone form of vitamin K to vitamin K epoxide.
  • 関連疾患Defects in GGCX are a cause of combined deficiency of vitamin K-dependent clotting factors type 1 (VKCFD1) [MIM:277450]; also known as multiple coagulation factor deficiency III (MCFD3). VKCFD leads to a bleeding tendency that is usually reversed by oral administration of vitamin K.
    Defects in GGCX are the cause of pseudoxanthoma elasticum-like disorder with multiple coagulation factor deficiency (PXEL-MCFD) [MIM:610842]. This syndrome is characterized by hyperlaxity of the skin involving the entire body. Important phenotypic differences with classical PXE include much more severe skin laxity with spreading toward the trunk and limbs with thick, leathery skin folds rather than confinement to flexural areas, and no decrease in visual acuity. Moreover, detailed electron microscopic analyzes revealed that alterations of elastic fibers as well as their mineralization are slightly different from those in classic PXE.
  • 配列類似性Belongs to the vitamin K-dependent gamma-carboxylase family.
  • 細胞内局在Endoplasmic reticulum membrane.
  • Information by UniProt
  • 参照データベース
  • 別名
    • FLJ26629 antibody
    • Gamma glutamyl carboxylase antibody
    • Gamma-glutamyl carboxylase antibody
    • GC antibody
    • GGCX antibody
    • Peptidyl glutamate 4 carboxylase antibody
    • Peptidyl-glutamate 4-carboxylase antibody
    • Vitamin K dependent gamma carboxylase antibody
    • Vitamin K gamma glutamyl carboxylase antibody
    • Vitamin K-dependent gamma-carboxylase antibody
    • VKCFD 1 antibody
    • VKCFD1 antibody
    • VKGC_HUMAN antibody
    see all

Anti-GGCX antibody 画像

  • Anti-GGCX antibody (ab106924) at 0.3 µg/ml (for 1 hour) + Human Muscle lysate in RIPA buffer at 35 µg
    Developed using the ECL technique

    Predicted band size : 88 kDa

Anti-GGCX antibody (ab106924) 使用論文

ab106924 has not yet been referenced specifically in any publications.

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