2.7.1.112; CEK2; HSFGFR3EX; fibroblast growth factor receptor 3 isoform 1 precursor; fibroblast growth factor receptor 3 isoform 2 precursor

For Research Use Only. Not for use in diagnostic procedures.

Polyclonal

Rabbit

The antibody detects endogenous levels of total FGFR3 protein.

The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.

Rabbit IgG in phosphate buffered saline (without Mg2+ and Ca2+), pH 7.4, 150mM NaCl, 0.02% sodium azide and 50% glycerol.

1.0 mg/ml (lot specific)

Store at -20 degree C

Small volumes of anti-FGFR3 antibody vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.

Tyrosine-protein kinase that acts as cell-surface receptor for fibroblast growth factors and plays an essential role in the regulation of cell proliferation, differentiation and apoptosis. Plays an essential role in the regulation of chondrocyte differentiation, proliferation and apoptosis, and is required for normal skeleton development. Regulates both osteogenesis and postnatal bone mineralization by osteoblasts. Promotes apoptosis in chondrocytes, but can also promote cancer cell proliferation. Required for normal development of the inner ear. Phosphorylates PLCG1, CBL and FRS2. 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. Plays a role in the regulation of vitamin D metabolism. Mutations that lead to constitutive kinase activation or impair normal FGFR3 maturation, internalization and degradation lead to aberrant signaling. Over-expressed or constitutively activated FGFR3 promotes activation of PTPN11/SHP2, STAT1, STAT5A and STAT5B. Secreted isoform 3 retains its capacity to bind FGF1 and FGF2 and hence may interfere with FGF signaling.Jing Chen, Blood, Jul 2005; 106: 328 - 337.Qing Zhang, Diabetes, Jan 2007; 56: 96 - 106.Nigel P. Pringle, Development, Jan 2003; 130: 93.Gladys Valverde-Franco, Hum. Mol. Genet., Feb 2004; 13: 271 - 284.

Total protein Ab

Western Blot (WB), Immunohistochemistry (IHC)

Western blotting: 1:500~1:3000
Immunohistochemistry: 1:50~1:100

Immunohistochemical analysis of paraffin-embedded human breast carcinoma tissue using FGFR3 antibody.

Western blot analysis of extracts from LOVO cells, using FGFR3 antibody.

Western blot analysis of extracts from 293 cells (Lane 2) and HepG2 cells (Lane 3), using FGFR3 antiobdy. The lane on the left is treated with synthesized peptide.

85,083 Da

fibroblast growth factor receptor 3

fibroblast growth factor receptor 3

Fibroblast growth factor receptor 3

JTK4; FGFR-3??[Similar Products]

FGFR3_HUMAN

This gene encodes a member of the fibroblast growth factor receptor (FGFR) family, with its amino acid sequence being highly conserved between members and among divergent species. FGFR family members differ from one another in their ligand affinities and tissue distribution. A full-length representative protein would consist of an extracellular region, composed of three immunoglobulin-like domains, a single hydrophobic membrane-spanning segment and a cytoplasmic tyrosine kinase domain. The extracellular portion of the protein interacts with fibroblast growth factors, setting in motion a cascade of downstream signals, ultimately influencing mitogenesis and differentiation. This particular family member binds acidic and basic fibroblast growth hormone and plays a role in bone development and maintenance. Mutations in this gene lead to craniosynostosis and multiple types of skeletal dysplasia. Three alternatively spliced transcript variants that encode different protein isoforms have been described. [provided by RefSeq, Jul 2009]

FGFR3: a receptor tyrosine kinase of the highly-conserved FGFR family that binds fibroblast growth factor (FGF). Mutations are associated with thanatophoric dysplasia (TD), craniosynostosis Adelaide type, many craniosynostotic syndromes and bone malformations. Three splice-variant isoforms have been described. Activating point mutations cause dwarfism, including achondroplasia, hypochrondroplasia and thanatophoric dysplasia, and facial and other morphogenetic disorders, including Crouzon syndrome, craniosynostosis Adelaide type, San Diego skeletal displasia and Muenke syndrome. Translocations t(4;14) involving the IgH region are common in multiple myeloma and frequently involve FGFR3. Activated FGFR3 found in 30% of bladder cancers and several cervical cancers, but not in other tumors. Two mutations found in colorectal cancer.

Protein type: Kinase, protein; EC 2.7.10.1; Protein kinase, TK; Protein kinase, tyrosine (receptor); Membrane protein, integral; TK group; FGFR family

Chromosomal Location of Human Ortholog: 4p16.3

Cellular Component: Golgi apparatus; internal side of plasma membrane; transport vesicle; focal adhesion; cell surface; endoplasmic reticulum; perinuclear region of cytoplasm; lysosome; integral to plasma membrane; plasma membrane; extracellular region; nucleus

Molecular Function: protein binding; fibroblast growth factor binding; fibroblast growth factor receptor activity; protein-tyrosine kinase activity; ATP binding

Biological Process: peptidyl-tyrosine phosphorylation; nerve growth factor receptor signaling pathway; somatic stem cell maintenance; protein amino acid autophosphorylation; negative regulation of transcription from RNA polymerase II promoter; bone mineralization; positive regulation of tyrosine phosphorylation of Stat3 protein; substantia nigra development; cell-cell signaling; positive regulation of MAPKKK cascade; inner ear receptor cell differentiation; positive regulation of neuron apoptosis; forebrain development; positive regulation of cell proliferation; chondrocyte differentiation; morphogenesis of an epithelium; response to axon injury; skeletal development; negative regulation of epithelial cell proliferation; endochondral ossification; epidermal growth factor receptor signaling pathway; negative regulation of developmental growth; fibroblast growth factor receptor signaling pathway; phosphoinositide-mediated signaling; myelination in the central nervous system; MAPKKK cascade; positive regulation of phosphoinositide 3-kinase activity; digestive tract morphogenesis; JAK-STAT cascade; positive regulation of tyrosine phosphorylation of Stat1 protein; positive regulation of protein ubiquitination; negative regulation of smoothened signaling pathway; negative regulation of mitosis; negative regulation of astrocyte differentiation; insulin receptor signaling pathway; innate immune response; lens morphogenesis in camera-type eye; positive regulation of endothelial cell proliferation; positive regulation of cell differentiation

Disease: Bladder Cancer; Achondroplasia; Hypochondroplasia; Muenke Syndrome; Achondroplasia, Severe, With Developmental Delay And Acanthosis Nigricans; Cervical Cancer; Thanatophoric Dysplasia, Type I; Colorectal Cancer; Crouzon Syndrome With Acanthosis Nigricans; Lacrimoauriculodentodigital Syndrome; Camptodactyly, Tall Stature, And Hearing Loss Syndrome; Thanatophoric Dysplasia, Type Ii; Testicular Germ Cell Tumor; Nevus, Epidermal

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