Anti-GGCX Antibody Picoband® Fluoro594 Conjugated

Gamma-glutamyl carboxylase is an enzyme that in humans is encoded by the GGCX gene, located on chromosome 2 at 2p12. 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.

Expressed in fibroblasts, heart, skeletal muscle, brain and pancreas. Expressed at an intermediate level in lung and kidney, and at a low level in liver and placenta. Expressed at a lower level in fibroblasts from hypertrophic scar lesional skin and in fibroblasts from patients with systemic sclerosis than in normal skin fibroblasts.

Seems to mediate adhesion by cell surface integrin binding. May serve as a communication link between the dermal fibroblast cell surface and its extracellular matrix environment. Enhances TGFB1 activity. Inhibits cell proliferation. Accelerates collagen fibril formation, and stabilizes collagen fibrils against low-temperature dissociation (By similarity) .

1. Brenner, B., Sanchez-Vega, B., Wu, S.-M., Lanir, N., Stafford, D. W., Solera, J. A missense mutation in a gamma-glutamyl carboxylase gene causes combined deficiency of all vitamin K-dependent blood coagulation factors. Blood 92: 4554-4559, 1998. 2. Brenner, B., Tavori, S., Zivelin, A., Keller, C. B., Suttie, J. W., Tatarsky, I., Seligsohn, U. Hereditary deficiency of all vitamin K-dependent procoagulants and anticoagulants. Brit. J. Haemat. 75: 537-542, 1990. 3. Kuo, W.-L., Stafford, D. W., Cruces, J., Gray, J., Solera, J. Chromosomal localization of the gamma-glutamyl carboxylase gene at 2p12. Genomics 25: 746-748, 1995.