Rabbit GGCX Polyclonal Antibody | anti-GGCX antibody

GGCX antibody - middle region

Cat. Num. AAA3207326

• Gene Names: GGCX; VKCFD1
• Reactivity: Cow, Dog, Guinea Pig, Horse, Human, Mouse, Rabbit, Rat, Sheep
• Applications: Immunohistochemistry, Western Blot
• Purity: Affinity Purified
• Synonyms: GGCX; Polyclonal Antibody; GGCX antibody - middle region; anti-GGCX antibody

• Host: Rabbit
• Reactivity: Cow, Dog, Guinea Pig, Horse, Human, Mouse, Rabbit, Rat, Sheep
• Clonality: Polyclonal
• Purity/Purification: Affinity Purified
• Form/Format: Liquid. Purified antibody supplied in 1x PBS buffer with 0.09% (w/v) sodium azide and 2% sucrose.
• Sequence: Synthetic peptide located within the following region: FLLRKLYVFRRSFLMTCISLRNLILGRPSLEQLAQEVTYANLRPFEAVGE
• Sequence Length: 758

• Applicable Applications for anti-GGCX antibody: Immunohistochemistry (IHC), Western Blot (WB)
• Homology: Cow: 100%; Dog: 100%; Guinea Pig: 100%; Horse: 100%; Human: 100%; Mouse: 100%; Rabbit: 100%; Rat: 100%; Sheep: 100%
• Immunogen: The immunogen is a synthetic peptide directed towards the middle region of human GGCX
• Preparation and Storage: For short term use, store at 2-8 degree C up to 1 week. For long term storage, store at -20 degree C in small aliquots to prevent freeze-thaw cycles.

Immunohistochemistry (IHC)

(Rabbit Anti-GGCX AntibodyFormalin Fixed Paraffin Embedded Tissue: Human Adult LiverObserved Staining: Cytoplasm in hepatocytes, strong signal, wide tissue distributionPrimary Antibody Concentration: 1:100Secondary Antibody: Donkey anti-Rabbit-Cy3Secondary Antibody Concentration: 1:200Magnification: 20XExposure Time: 0.5 - 2.0 secProtocol located in Reviews and Data.)

Western Blot (WB)

(Host: RabbitTarget Name: GGCXSample Type: JurkatAntibody Dilution: 1.0ug/mlGGCX is strongly supported by BioGPS gene expression data to be expressed in Human Jurkat cells)

Western Blot (WB)

(WB Suggested Anti-GGCX Antibody Titration: 0.2-1 ug/mlELISA Titer: 1:1562500Positive Control: MCF7 cell lysate.GGCX is strongly supported by BioGPS gene expression data to be expressed in MCF7)

Related Product Information for anti-GGCX antibody

This is a rabbit polyclonal antibody against GGCX. It was validated on Western Blot using a cell lysate as a positive control.

Target Description: GGCX is 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.Gamma-glutamyl carboxylase accomplishes the posttranslational modification required for the activity of all of the vitamin K-dependent proteins (Wu et al., 1991 [PubMed 1749935]). These include some of the blood coagulation and anticoagulation proteins as well as bone gamma-carboxyglutamic acid (Gla) protein (BGLAP; MIM 112260) and bone matrix protein (MGP; MIM 154870).[supplied by OMIM]. Publication Note: This RefSeq record includes a subset of the publications that are available for this gene. Please see the Entrez Gene record to access additional publications.

Product Categories/Family for anti-GGCX antibody

Polyclonal; Membrane Protein; Drugs and Drug Metabolism; Disease Related

NCBI and Uniprot Product Information

• NCBI GI #: 21361163
• NCBI GeneID: 2677
• NCBI Accession #: NP_000812
• NCBI GenBank Nucleotide #: NM_000821
• UniProt Accession #: P38435
• Molecular Weight: 87kDa
• NCBI Official Full Name: vitamin K-dependent gamma-carboxylase isoform 1
• NCBI Official Synonym Full Names: gamma-glutamyl carboxylase
• NCBI Official Symbol: GGCX
• NCBI Official Synonym Symbols: VKCFD1
• NCBI Protein Information: vitamin K-dependent gamma-carboxylase
• UniProt Protein Name: Vitamin K-dependent gamma-carboxylase
• Protein Family: Vitamin K-dependent gamma-carboxylase
• UniProt Gene Name: GGCX
• UniProt Synonym Gene Names: GC
• UniProt Entry Name: VKGC_HUMAN

NCBI Description

This gene encodes an integral membrane protein of the rough endoplasmic reticulum that carboxylates glutamate residues of vitamin K-dependent proteins to gamma carboxyl glutamate, a modification that is required for their activity. The vitamin K-dependent protein substrates have a propeptide that binds the enzyme, with carbon dioxide, dioxide, and reduced vitamin K acting as co-substrates. Vitamin K-dependent proteins affect a number of physiologic processes including blood coagulation, prevention of vascular calcification, and inflammation. Allelic variants of this gene have been associated with pseudoxanthoma elasticum-like disorder with associated multiple coagulation factor deficiency. Alternative splicing results in multiple transcript variants. [provided by RefSeq, Aug 2015]

Uniprot Description

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

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