Overview

  • Product name

  • Description

    Goat polyclonal to GGCX
  • Host species

    Goat
  • Specificity

    This antibody is expected to recognize both isoforms (NP_000812.2; NP_001135741.1).
  • Tested applications

    Suitable for: WBmore details
  • Species reactivity

    Reacts with: Human
    Predicted to work with: Dog, Orangutan
  • Immunogen

    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).

  • Positive control

    • Human Muscle lysates.

Properties

  • Form

    Liquid
  • Storage instructions

    Shipped at 4°C. Upon delivery aliquot and store at -20°C. Avoid freeze / thaw cycles.
  • Storage buffer

    Preservative: 0.02% Sodium Azide
    Constituents: 0.5% BSA, Tris buffered saline, pH 7.3
  • Concentration information loading...
  • Purity

    Immunogen affinity purified
  • Purification notes

    Purified from goat serum by ammonium sulphate precipitation followed by antigen affinity chromatography using the immunizing peptide.
  • Clonality

    Polyclonal
  • Isotype

    IgG
  • Research areas

Applications

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.

Application Abreviews Notes
WB Use a concentration of 0.3 - 1 µg/ml. Predicted molecular weight: 88 kDa.

Target

  • Function

    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.
  • Involvement in disease

    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.
  • Sequence similarities

    Belongs to the vitamin K-dependent gamma-carboxylase family.
  • Cellular localization

    Endoplasmic reticulum membrane.
  • Information by UniProt
  • Database links

  • Alternative names

    • 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

Images

  • 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

References

ab106924 has not yet been referenced specifically in any publications.

Customer reviews and Q&As

There are currently no Customer reviews or Questions for ab106924.
Please use the links above to contact us or submit feedback about this product.

Please note: All products are "FOR RESEARCH USE ONLY. NOT FOR USE IN DIAGNOSTIC PROCEDURES"
For licensing inquiries, please contact partnerships@abcam.com

Sign up