Recombinant Human GGCX protein (ab114658)
Key features and details
- Expression system: Wheat germ
- Suitable for: WB, SDS-PAGE, ELISA
Description
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Product name
Recombinant Human GGCX protein -
Expression system
Wheat germ -
Accession
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Protein length
Protein fragment -
Animal free
No -
Nature
Recombinant -
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Species
Human -
Sequence
ADFPGLHLENFVSEDLGNTSIQLLQGEVTVELVAEQKNQTLREGEKMQLP AGEYHKVYTTSPSPSCYMYVYVNTTELALEQDLAYLQELKEKVENGS -
Predicted molecular weight
36 kDa including tags -
Amino acids
533 to 629
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Specifications
Our Abpromise guarantee covers the use of ab114658 in the following tested applications.
The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
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Applications
Western blot
SDS-PAGE
ELISA
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Form
Liquid -
Concentration information loading...
Preparation and Storage
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Stability and Storage
Shipped on dry ice. Upon delivery aliquot and store at -80ºC. Avoid freeze / thaw cycles.
pH: 8.00
Constituents: 0.3% Glutathione, 0.79% Tris HCl
General Info
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Alternative names
- FLJ26629
- Gamma glutamyl carboxylase
- Gamma-glutamyl carboxylase
see all -
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
Images
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SDS-PAGE - Recombinant Human GGCX protein (ab114658)SDS-PAGE analysis of ab114658 on a 12.5% gel stained with Coomassie Blue.
Protocols
To our knowledge, customised protocols are not required for this product. Please try the standard protocols listed below and let us know how you get on.
Datasheets and documents
References (0)
ab114658 has not yet been referenced specifically in any publications.
