Recombinant Human GGCX protein (ab114658)

Overview

  • Product nameRecombinant Human GGCX protein
  • Protein lengthProtein fragment

Description

  • NatureRecombinant
  • SourceWheat germ
  • Amino Acid Sequence
    • AccessionP38435
    • SpeciesHuman
    • SequenceADFPGLHLENFVSEDLGNTSIQLLQGEVTVELVAEQKNQTLREGEKMQLP AGEYHKVYTTSPSPSCYMYVYVNTTELALEQDLAYLQELKEKVENGS
    • Molecular weight36 kDa including tags
    • Amino acids533 to 629

Associated products

Specifications

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.

  • Applications

    Western blot

    SDS-PAGE

    ELISA

  • FormLiquid
  • Additional notesProtein concentration is above or equal to 0.05 mg/ml.
    Best used within three months from the date of receipt.
  • Concentration information loading...

Preparation and Storage

  • Stability and Storage

    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

General Info

  • Alternative names
    • 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
  • FunctionMediates 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.
  • Involvement in diseaseDefects 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.
  • Sequence similaritiesBelongs to the vitamin K-dependent gamma-carboxylase family.
  • Cellular localizationEndoplasmic reticulum membrane.
  • Information by UniProt

Recombinant Human GGCX protein images

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

References for Recombinant Human GGCX protein (ab114658)

ab114658 has not yet been referenced specifically in any publications.

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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"