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Mouse Anti-GGCX Recombinant Antibody (CBLG1-090) (CBMAB-G0419-LY)

This product is antibody recognizes GGCX. The antibody CBLG1-090 immunoassay techniques such as: ELISA, IHC, WB.
See all GGCX antibodies

Summary

Host Animal
Mouse
Specificity
Human
Clone
CBLG1-090
Antibody Isotype
IgG1
Application
ELISA, IHC, WB

Basic Information

Specificity
Human
Antibody Isotype
IgG1
Clonality
Monoclonal
Application Notes
The COA includes recommended starting dilutions, optimal dilutions should be determined by the end user.

Formulations & Storage [For reference only, actual COA shall prevail!]

Format
Liquid
Preservative
0.09% sodium azide
Purity
> 95% Purity determined by SDS-PAGE.
Storage
Store at +4°C short term (1-2 weeks). Aliquot and store at -20°C long term. Avoid repeated freezethaw cycles.

Target

Full Name
Gamma-Glutamyl Carboxylase
Introduction
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]
Entrez Gene ID
2677
UniProt ID
P38435
Alternative Names
Gamma-Glutamyl Carboxylase; Vitamin K-Dependent Gamma-Carboxylase; Peptidyl-Glutamate 4-Carboxylase; Vitamin K Gamma Glutamyl Carboxylase; EC 4.1.1.90; VKCFD1; GC;
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.
Biological Process
Blood coagulation Source: ProtInc
Cellular protein modification process Source: ProtInc
Peptidyl-glutamic acid carboxylation Source: GO_Central
Cellular Location
Endoplasmic reticulum membrane
Involvement in disease
Combined deficiency of vitamin K-dependent clotting factors 1 (VKCFD1):
VKCFD leads to a bleeding tendency that is usually reversed by oral administration of vitamin K.
Pseudoxanthoma elasticum-like disorder with multiple coagulation factor deficiency (PXEL-MCFD):
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.
Topology
Cytoplasmic: 2-60
Helical: 61-81
Lumenal: 82-113
Helical: 114-134
Cytoplasmic: 135-136
Helical: 137-157
Lumenal: 158-292
Helical: 293-313
Cytoplasmic: 314-361
Helical: 362-382
Lumenal: 383-758
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For research use only. Not intended for any clinical use.

Custom Antibody Labeling

We also offer labeled antibodies developed using our catalog antibody products and nonfluorescent conjugates (HRP, AP, Biotin, etc.) or fluorescent conjugates (Alexa Fluor, FITC, TRITC, Rhodamine, Texas Red, R-PE, APC, Qdot Probes, Pacific Dyes, etc.).

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