- Catalog number
- PA1477-carrier-free
- Size / count
- 100 µg/Vial
- Dry ice
- No
- Hazardous
- No
- Discontinued
- No
- Background
- FGFR1, Fibroblast growth factor receptor 1, also known as basic fibroblast growth factor receptor 1, fms-related tyrosine kinase-2/Pfeiffer syndrome, and CD331, is a receptor tyrosine kinase whose ligands are specific members of the fibroblast growth factor family. The FGFR1 gene is localized to 8p12-p11.2 by in situ hybridization. FGFR1 is essential for the normal formation of the organ of Corti and that phenotype severity observed in FGFR1 mutants is dependent on the dose of FGFR1.Mutations in this gene have been associated with Pfeiffer syndrome, Jackson-Weiss syndrome, Antley-Bixler syndrome, osteoglophonic dysplasia, squamous cell lung cancer and autosomal dominant Kallmann syndrome 2.
- Synonyms
- Fibroblast growth factor receptor 1;FGFR-1;2.7.10.1;Basic fibroblast growth factor receptor 1;BFGFR;bFGF-R-1;Fms-like tyrosine kinase 2;FLT-2;N-sam;Proto-oncogene c-Fgr;CD331;FGFR1;BFGFR, CEK, FGFBR, FLG, FLT2, HBGFR;
- Gene Name
- Fibroblast growth factor receptor 1
- UniProt
- P11362
- Host
- Rabbit
- Reactivity
- Human,Mouse,Rat
- Cross Reactivity
- No cross-reactivity with other proteins
- Immunogen
- A synthetic peptide corresponding to a sequence at the C-terminus of human FGFR1, different from the related mouse sequence by two amino acids and rat sequence by three amino acids.
- Clonality
- Polyclonal
- Tissue Specificity
- Detected in astrocytoma, neuroblastoma and adrenal cortex cell lines. Some isoforms are detected in foreskin fibroblast cell lines, however isoform 17, isoform 18 and isoform 19 are not detected in these cells. .
- Applications
- IHC,WB
- Purification
- Immunogen affinity purified.
- Concentration
- Adding 0.2 ml of distilled water will yield a concentration of 500 μg/ml.
- Form
- Lyophilized
- Reconstitution
- Add 0.2ml of distilled water will yield a concentration of 500ug/ml.
- Function
- Tyrosine-protein kinase that acts as cell-surface receptor for fibroblast growth factors and plays an essential role in the regulation of embryonic development, cell proliferation, differentiation and migration. Required for normal mesoderm patterning and correct axial organization during embryonic development, normal skeletogenesis and normal development of the gonadotropin-releasing hormone (GnRH) neuronal system. Phosphorylates PLCG1, FRS2, GAB1 and SHB. Ligand binding leads to the activation of several signaling cascades. Activation of PLCG1 leads to the production of the cellular signaling molecules diacylglycerol and inositol 1,4,5-trisphosphate. Phosphorylation of FRS2 triggers recruitment of GRB2, GAB1, PIK3R1 and SOS1, and mediates activation of RAS, MAPK1/ERK2, MAPK3/ERK1 and the MAP kinase signaling pathway, as well as of the AKT1 signaling pathway. Promotes phosphorylation of SHC1, STAT1 and PTPN11/SHP2. In the nucleus, enhances RPS6KA1 and CREB1 activity and contributes to the regulation of transcription. FGFR1 signaling is down-regulated by IL17RD/SEF, and by FGFR1 ubiquitination, internalization and degradation. .
- Components
- Each vial contains 5mg BSA, 0.9mg NaCl, 0.2mg Na2HPO4, 0.05mg Thimerosal, 0.05mg NaN3.
- References & Citations
- 1. Pirvola, U., Ylikoski, J., Trokovic, R., Hebert, J. M., McConnell, S. K., Partanen, J. FGFR1 is required for the development of the auditory sensory epithelium.
2. Roscioli, T., Flanagan, S., Kumar, P., Masel, J., Gattas, M., Hyland, V. J., Glass, I. A.Clinical findings in a patient with FGFR1 P252R mutation and comparison with the literature.Am. J. Med. Genet. 93: 22-28, 2000.
3. Ruta, M., Howk, R., Ricca, G., Drohan, W., Zabelshansky, M., Laureys, G., Barton, D. E., Francke, U., Schlessinger, J., Givol, D.A novel protein tyrosine kinase gene whose expression is modulated during endothelial cell differentiation.Oncogene 3: 9-15, 1988.
- Storage Conditions
- Store at -20℃ for one year from date of receipt. After reconstitution, at 4℃ for one month. It can also be aliquotted and stored frozen at -20℃ for six months. Avoid repeated freeze-thaw cycles.
- Calculated Molecular Weight
- 91868 MW
- Observed Molecular Weight
- 24 kDa
- Fragment
- Rabbit IgG
- Specificity
- No cross reactivity with other proteins.
- Applications Notes
- Immunohistochemistry (Paraffin-embedded Section), 0.5-1μg/ml, Human, Rat, Mouse, By Heat<br>Western blot, 0.1-0.5μg/ml, Human, Rat, Mouse<br>
- Subcellular Location
- Cell membrane; Single-pass type I membrane protein. Nucleus. Cytoplasm, cytosol. Cytoplasmic vesicle. After ligand binding, both receptor and ligand are rapidly internalized. Can translocate to the nucleus after internalization, or by translocation from the endoplasmic reticulum or Golgi apparatus to the cytosol, and from there to the nucleus.
- Sequence Similarities
- Belongs to the protein kinase superfamily. Tyr protein kinase family. Fibroblast growth factor receptor subfamily.
- Protein Name
- Fibroblast growth factor receptor 1(FGFR-1/bFGF-R-1)
