GGCX, Polyclonal Antibody

Vitamin K-dependent gamma-carboxylase; Gamma-glutamyl carboxylase; Peptidyl-glutamate 4-carboxylase; Vitamin K gamma glutamyl carboxylase; GGCX; GC

For Research Use Only. Not for use in diagnostic procedures.

Polyclonal

Rabbit Ig

Rabbit

This GGCX antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide between 548-578 amino acids from the Central region of human GGCX.

Peptide Affinity Purified Rabbit Polyclonal Antibody (Pab)

Purified polyclonal antibody supplied in PBS with 0.09% (W/V) sodium azide. This antibody is purified through a protein A column, followed by peptide affinity purification.

Vial Concentration: 0.49 (lot specific)

Maintain refrigerated at 2-8 degree C for up to 6 months. For long term storage store at -20 degree C in small aliquots to prevent freeze-thaw cycles.

Small volumes of anti-GGCX antibody vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.

This gene encodes an enzyme which catalyzes the
posttranslational modification of vitamin K-dependent protein. Many
of these vitamin K-dependent proteins are involved in coagulation
so the function of the encoded enzyme is essential for hemostasis.
Mutations in this gene are associated with vitamin K-dependent
coagulation defect and PXE-like disorder with multiple coagulation
factor deficiency. Multiple transcript variants encoding different
isoforms have been found for this gene.

Cardiovascular

ELISA (EIA), Immunohistochemistry (IHC), Western Blot (WB)

WB~~1:1000

GGCX Antibody (Center) western blot analysis in 293 cell line lysates (35ug/lane).This demonstrates the GGCX antibody detected the GGCX protein (arrow).

GGCX Antibody (Center) immunohistochemistry analysis in formalin fixed and paraffin embedded human liver tissue followed by peroxidase conjugation of the secondary antibody and DAB staining.This data demonstrates the use of GGCX Antibody (Center) for immunohistochemistry. Clinical relevance has not been evaluated.

87561

gamma-glutamyl carboxylase

vitamin K-dependent gamma-carboxylase

Vitamin K-dependent gamma-carboxylase

GC  [Similar Products]

VKGC_HUMAN

This gene encodes an enzyme which catalyzes the posttranslational modification of vitamin K-dependent protein. Many of these vitamin K-dependent proteins are involved in coagulation so the function of the encoded enzyme is essential for hemostasis. Mutations in this gene are associated with vitamin K-dependent coagulation defect and PXE-like disorder with multiple coagulation factor deficiency. Multiple transcript variants encoding different isoforms have been found for this gene.[provided by RefSeq, Nov 2008]

GGCX: 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. Defects in GGCX are a cause of combined deficiency of vitamin K-dependent clotting factors type 1 (VKCFD1); 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). 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. Belongs to the vitamin K-dependent gamma-carboxylase family.

Protein type: Membrane protein, multi-pass; Lyase; Membrane protein, integral; EC 4.1.1.90; Ligase

Chromosomal Location of Human Ortholog: 2p12

Cellular Component: endoplasmic reticulum membrane; membrane; integral to membrane

Molecular Function: gamma-glutamyl carboxylase activity

Biological Process: cellular protein metabolic process; protein modification process; post-translational protein modification; blood coagulation; peptidyl-glutamic acid carboxylation

Disease: Pseudoxanthoma Elasticum-like Disorder With Multiple Coagulation Factor Deficiency; Vitamin K-dependent Clotting Factors, Combined Deficiency Of, 1

Higgins-Gruber, S.L., et al. J. Biol. Chem. 285(41):31502-31508(2010)
King, C.R., et al. Thromb. Haemost. 104(4):750-754(2010)
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Ikejiri, M., et al. Int. J. Hematol. 92(2):302-305(2010)
Schelleman, H., et al. Br J Clin Pharmacol 70(3):393-399(2010)

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