Overview

  • Product name

    Anti-GGCX antibody - C-terminal
    See all GGCX primary antibodies
  • Description

    Goat polyclonal to GGCX - C-terminal
  • Host species

    Goat
  • Tested applications

    Suitable for: WB, IHC-Pmore details
  • Species reactivity

    Reacts with: Human
    Predicted to work with: Monkey, Gorilla, Common marmoset, Orangutan
  • Immunogen

    Synthetic peptide corresponding to Human GGCX aa 746-757 (C terminal) (Cysteine residue). (NP_000812.2).
    Sequence:

    PPESNPDPVHSE


    Database link: P38435

  • Positive control

    • Human kidney tissue; Human muscle lysate.

Properties

  • Form

    Liquid
  • Storage instructions

    Shipped at 4°C. Store at +4°C short term (1-2 weeks). Upon delivery aliquot. Store at -20°C long term. Avoid freeze / thaw cycle.
  • Storage buffer

    pH: 7.30
    Preservative: 0.02% Sodium azide
    Constituents: 0.5% BSA, 99% Tris buffered saline
  • Concentration information loading...
  • Purity

    Immunogen affinity purified
  • Clonality

    Polyclonal
  • Isotype

    IgG
  • Research areas

Applications

Our Abpromise guarantee covers the use of ab219757 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. Detects a band of approximately 85 kDa (predicted molecular weight: 88 kDa).
IHC-P Use a concentration of 5 µg/ml. Perform heat mediated antigen retrieval before commencing with IHC staining protocol.

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

  • Immunohistochemical analysis of formalin-fixed, paraffin-embedded human kidney tissue labeling GGCX with ab219757 at 5 µg/ml.

  • Anti-GGCX antibody - C-terminal (ab219757) at 0.3 µg/ml + Human muscle lysate (in RIPA buffer) at 35 µg

    Developed using the ECL technique.

    Predicted band size: 88 kDa
    Observed band size: 85 kDa
    why is the actual band size different from the predicted?



    Primary incubation was 1 hour.

References

ab219757 has not yet been referenced specifically in any publications.

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There are currently no Customer reviews or Questions for ab219757.
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