- Catalog number
- A02643-1
- Size / count
- 100 µg/Vial
- Dry ice
- No
- Hazardous
- No
- Discontinued
- No
- Background
- 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.
- Synonyms
- Dermatopontin; Tyrosine-rich acidic matrix protein; TRAMP; DPT
- Gene Name
- GGCX
- Gene ID
- 2677
- UniProt
- P38435
- Host
- Rabbit
- Reactivity
- Human, Mouse
- Cross Reactivity
- No cross-reactivity with other proteins.
- Immunogen
- E.coli-derived human GGCX recombinant protein (Position: M75-A730) .
- Clonality
- Polyclonal
- Tissue Specificity
- 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.
- Applications
- ELISA, Flow Cytometry, IF, IHC, ICC, WB
- Field of Research
- Biochemicals, Chemical Type, Signal Transduction
- Purification
- Immunogen affinity purified.
- Concentration
- Adding 0.2 ml of distilled water will yield a concentration of 500 μg/ml.
- Form
- Lyophilized
- Reconstitution
- Adding 0.2 ml of distilled water will yield a concentration of 500 μg/ml.
- Function
- 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) .
- References & Citations
- 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.
- Storage Conditions
- At -20°C for one year from date of receipt. After reconstitution, at 4°C for one month. It can also be aliquotted and stored frozen at -20°C for six months. Avoid repeated freezing and thawing.
- Calculated Molecular Weight
- 33310 MW
- Observed Molecular Weight
- 88 kDa
- Applications Notes
- 6
- Gene Name Synonym
- Dermatopontin
- Subcellular Location
- Secreted, extracellular space, extracellular matrix.
- Isotype
- Rabbit IgG
- Contents
- Each vial contains 4 mg Trehalose, 0.9 mg NaCl, 0.2 mg Na2HPO4.