概述

  • 产品名称Anti-GGCX抗体
    参阅全部 GGCX 一抗
  • 描述
    山羊多克隆抗体to GGCX
  • 特异性This antibody is expected to recognize both isoforms (NP_000812.2; NP_001135741.1).
  • 经测试应用适用于: WBmore details
  • 种属反应性
    与反应: Human
    预测可用于: 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).

  • 阳性对照
    • Human Muscle lysates.

性能

  • 形式Liquid
  • 存放说明Shipped at 4°C. Upon delivery aliquot and store at -20°C. Avoid freeze / thaw cycles.
  • 存储溶液Preservative: 0.02% Sodium Azide
    Constituents: 0.5% BSA, Tris buffered saline, pH 7.3
  • Concentration information loading...
  • 纯度Immunogen affinity purified
  • 纯化说明Purified from goat serum by ammonium sulphate precipitation followed by antigen affinity chromatography using the immunizing peptide.
  • 克隆多克隆
  • 同种型IgG
  • 研究领域

应用

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.

应用 Ab评论 说明
WB Use a concentration of 0.3 - 1 µg/ml. Predicted molecular weight: 88 kDa.

靶标

  • 功能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.
  • 疾病相关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.
  • 序列相似性Belongs to the vitamin K-dependent gamma-carboxylase family.
  • 细胞定位Endoplasmic reticulum membrane.
  • Information by UniProt
  • 数据库链接
  • 别名
    • 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 图像

  • 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)参考文献

ab106924 has not yet been referenced specifically in any publications.

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