Recombinant人GGCX protein (ab114658)

概述

  • 产品名称Recombinant人GGCX protein
  • 蛋白长度Protein fragment

描述

  • 性质Recombinant
  • 来源Wheat germ
  • 氨基酸序列
    • AccessionP38435
    • 种属Human
    • 序列ADFPGLHLENFVSEDLGNTSIQLLQGEVTVELVAEQKNQTLREGEKMQLP AGEYHKVYTTSPSPSCYMYVYVNTTELALEQDLAYLQELKEKVENGS
    • 分子量36 kDa including tags
    • 氨基酸533 to 629

相关产品

技术指标

Our Abpromise guarantee covers the use of ab114658 in the following tested applications.

The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.

  • 应用

    Western blot

    SDS-PAGE

    ELISA

  • 形式Liquid
  • 补充说明Protein concentration is above or equal to 0.05 mg/ml.
    Best used within three months from the date of receipt.
  • Concentration information loading...

制备和贮存

  • 稳定性和存储

    Shipped on dry ice. Upon delivery aliquot and store at -80ºC. Avoid freeze / thaw cycles.

    pH: 8.00
    Constituents: 0.3% Glutathione, 0.79% Tris HCl

常规信息

  • 别名
    • FLJ26629
    • Gamma glutamyl carboxylase
    • Gamma-glutamyl carboxylase
    • GC
    • GGCX
    • Peptidyl glutamate 4 carboxylase
    • Peptidyl-glutamate 4-carboxylase
    • Vitamin K dependent gamma carboxylase
    • Vitamin K gamma glutamyl carboxylase
    • Vitamin K-dependent gamma-carboxylase
    • VKCFD 1
    • VKCFD1
    • VKGC_HUMAN
    see all
  • 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

Recombinant Human GGCX protein 图像

  • SDS-PAGE analysis of ab114658 on a 12.5% gel stained with Coomassie Blue.

Recombinant Human GGCX protein (ab114658)参考文献

ab114658 has not yet been referenced specifically in any publications.

Product Wall

Vielen Dank für Ihren Anruf.

Ich habe mit der Produktmanagerin gesprochen, leider haben wir jedoch kein anderes GGCXProtein zur Zeit. Wir werden versuchen, eines hinzuzufügen, jedoch wird das noch eine Weile dauern.

Da nu...

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