Rabbit anti-Human GGCX Polyclonal Antibody | anti-GGCX antibody

GGCX Antibody

Cat. Num. AAA9413713

• Gene Names: GGCX; VKCFD1
• Reactivity: Human
• Applications: Western Blot, Immunohistochemistry
• Purity: Antigen affinity purification.
• Synonyms: GGCX; Polyclonal Antibody; GGCX Antibody; VKCFD1; anti-GGCX antibody

• Host: Rabbit
• Reactivity: Human
• Clonality: Polyclonal
• Specificity: The antibody detects endogenous levels of total GGCX protein.
• Purity/Purification: Antigen affinity purification.
• Form/Format: Rabbit IgG in pH7.3 PBS, 0.05% NaN3, 50% Glycerol.
• Concentration: 2.2 mg/ml (varies by lot)
• Sequence Length: 758

• Applicable Applications for anti-GGCX antibody: Western Blot (WB), Immunohistochemistry (IHC)
• Application Notes: Western blotting: 1:500-1:2000; Immunohistochemistry: 1:50-1:200
• Immunogen Type: Recombinant Protein
• Immunogen Description: Fusion protein corresponding to residues near the C terminal of human gamma-glutamyl carboxylase
• Target Name: GGCX
• Preparation and Storage: Store at -20 degree C

Testing Data

(Gel: 6%SDS-PAGE Lysates (from left to right): MCF7, hela and 293T cell Amount of lysate: 40ug per lane Primary antibody: 1/400 dilution Secondary antibody dilution: 1/8000 Exposure time: 40 seconds)

Immunohistochemistry (IHC)

(Immunohistochemical analysis of paraffin-embedded Human liver cancer tissue using at dilution 1/50.)

Related Product Information for anti-GGCX antibody

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.

Product Categories/Family for anti-GGCX antibody

Total protein Ab

NCBI and Uniprot Product Information

• NCBI GI #: 21361163
• NCBI GeneID: 2677
• NCBI Accession #: NP_000812.2
• NCBI GenBank Nucleotide #: NM_000821.6
• UniProt Accession #: P38435; Q14415; Q6GU45; B4DMC5; E9PEE1
• Molecular Weight: 80,989 Da
• 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; Membrane protein, integral; Ligase; Membrane protein, multi-pass; 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; blood coagulation; post-translational protein modification; peptidyl-glutamic acid carboxylation

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