Rabbit GGCX Polyclonal Antibody | anti-GGCX antibody
GGCX Antibody BIOTIN-Conjugated
ELISA: 1:10,000
Immunoprecipitation: 1:150
Western Blot: 1:500
Mediates the vitamin K-dependent carboxylation of glutatmate residues to calcium-binding gamma-carboxyglutamate residues with the concomitant converstion of the reduced hydroquinone form of vitamin K to vitamin K epoxide.
NCBI and Uniprot Product Information
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: Ligase; Membrane protein, integral; Membrane protein, multi-pass; EC 4.1.1.90; Lyase
Chromosomal Location of Human Ortholog: 2p12
Cellular Component: endoplasmic reticulum membrane; integral to membrane; membrane
Molecular Function: gamma-glutamyl carboxylase activity
Biological Process: blood coagulation; peptidyl-glutamic acid carboxylation; protein modification process
Disease: Pseudoxanthoma Elasticum-like Disorder With Multiple Coagulation Factor Deficiency; Vitamin K-dependent Clotting Factors, Combined Deficiency Of, 1
Research Articles on GGCX
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Product Notes
The GGCX ggcx (Catalog #AAA541682) is an Antibody produced from Rabbit and is intended for research purposes only. The product is available for immediate purchase. The GGCX Antibody BIOTIN-Conjugated reacts with Human, Mouse, Rat and may cross-react with other species as described in the data sheet. AAA Biotech's GGCX can be used in a range of immunoassay formats including, but not limited to, ELISA (EIA), IMM, Western Blot (WB). Dot Blot: 1:10,000 ELISA: 1:10,000 Immunoprecipitation: 1:150 Western Blot: 1:500. Researchers should empirically determine the suitability of the GGCX ggcx for an application not listed in the data sheet. Researchers commonly develop new applications and it is an integral, important part of the investigative research process. It is sometimes possible for the material contained within the vial of "GGCX, Polyclonal Antibody" to become dispersed throughout the inside of the vial, particularly around the seal of said vial, during shipment and storage. We always suggest centrifuging these vials to consolidate all of the liquid away from the lid and to the bottom of the vial prior to opening. Please be advised that certain products may require dry ice for shipping and that, if this is the case, an additional dry ice fee may also be required.Precautions
All products in the AAA Biotech catalog are strictly for research-use only, and are absolutely not suitable for use in any sort of medical, therapeutic, prophylactic, in-vivo, or diagnostic capacity. By purchasing a product from AAA Biotech, you are explicitly certifying that said products will be properly tested and used in line with industry standard. AAA Biotech and its authorized distribution partners reserve the right to refuse to fulfill any order if we have any indication that a purchaser may be intending to use a product outside of our accepted criteria.Disclaimer
Though we do strive to guarantee the information represented in this datasheet, AAA Biotech cannot be held responsible for any oversights or imprecisions. AAA Biotech reserves the right to adjust any aspect of this datasheet at any time and without notice. It is the responsibility of the customer to inform AAA Biotech of any product performance issues observed or experienced within 30 days of receipt of said product. To see additional details on this or any of our other policies, please see our Terms & Conditions page.Item has been added to Shopping Cart
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