catalog number :
MBS9600098
products full name :
FGFR3 Antibody
products short name :
[FGFR3]
products name syn :
[ACH; CD 333; CD333; CD333 antigen; CEK 2; CEK2; FGFR 3; FGFR-3; FGFR3; FGFR3_HUMAN; Fibroblast growth factor receptor 3 (achondroplasia thanatophoric dwarfism); Fibroblast growth factor receptor 3; Heparin binding growth factor receptor; HSFGFR3EX; Hydroxyaryl protein kinase; JTK 4; JTK4; MFR 3; SAM 3; Tyrosine kinase JTK 4; Tyrosine kinase JTK4; Z FGFR 3]
other names :
[fibroblast growth factor receptor 3 isoform 1; Fibroblast growth factor receptor 3; fibroblast growth factor receptor 3; fibroblast growth factor receptor 3; CD_antigen: CD333]
products gene name :
[FGFR3]
other gene names :
[FGFR3; FGFR3; ACH; CEK2; JTK4; CD333; HSFGFR3EX; JTK4; FGFR-3]
reactivity :
Human, Mouse, Rat
specificity :
FGFR3 antibody detects endogenous levels of total FGFR3
purity :
The antiserum was purified by peptide affinity chromatography using SulfoLink™ Coupling Resin.
form :
Rabbit IgG in phosphate buffered saline , pH 7.4, 150mM NaCl, 0.02% sodium azide and 50% glycerol.
storage stability :
Store at -20°C. Stable for 12 months from date of receipt.
tested application :
Western Blot (WB), Immunohistochemistry (IHC), ELISA (EIA)
app notes :
WB: 1:500-1:3000. IHC: 1:50-1:200. IF/ICC: 1:100-1:500
image1 heading :
Immunofluorescence (IF)
image2 heading :
Western Blot (WB)
image3 heading :
Immunohistochemistry (IHC)
other info1 :
Immunogen: A synthesized peptide derived from human FGFR3, corresponding to a region within C-terminal amino acids. Subcellular Location: Membrane. Tissue Specificity: Expressed in brain, kidney and testis. Very low or no expression in spleen, heart, and muscle. In 20- to 22-week old fetuses it is expressed at high level in kidney, lung, small intestine and brain, and to a lower degree in spleen, liver, and muscle. Isoform 2 is detected in epithelial cells. Isoform 1 is not detected in epithelial cells. Isoform 1 and isoform 2 are detected in fibroblastic cells.
other info2 :
Similarity: The second and third Ig-like domains directly interact with fibroblast growth factors (FGF) and heparan sulfate proteoglycans.Belongs to the protein kinase superfamily. Tyr protein kinase family. Fibroblast growth factor receptor subfamily.
products description :
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.
ncbi acc num :
NP_000133.1
ncbi gb acc num :
NM_000142.4
ncbi pathways :
Adaptive Immune System Pathway (366160); Bladder Cancer Pathway (83115); Bladder Cancer Pathway (527); Constitutive PI3K/AKT Signaling In Cancer Pathway (685535); DAP12 Interactions Pathway (685549); DAP12 Signaling Pathway (685550); Disease Pathway (530764); Downstream Signaling Events Of B Cell Receptor (BCR) Pathway (576250); Downstream Signal Transduction Pathway (106385); Downstream Signaling Of Activated FGFR Pathway (160957)
ncbi summary :
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. [provided by RefSeq, Aug 2017]
uniprot summary :
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.