catalog number :
MBS142778
products type :
Recombinant Protein
products full name :
Recombinant Human Fibroblast Growth Factor Receptor-3
products short name :
Fibroblast Growth Factor Receptor-3
products name syn :
FGFR3 Human; Fibroblast Growth Factor Receptor 3 Fc Chimera Human Recombinant; Achondroplasia; Thanatophoric Dwarfism; CD333; ACH; CEK2; JTK4; HSFGFR3EX; FGFR3
other names :
fibroblast growth factor receptor 3 isoform 1; Fibroblast growth factor receptor 3; fibroblast growth factor receptor 3; FGFR-3; fibroblast growth factor receptor 3 variant 4; hydroxyaryl-protein kinase; tyrosine kinase JTK4; 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
uniprot entry name :
FGFR3_HUMAN
sequence :
ESLGTEQRVV GRAAEVPGPE PGQQEQLVFG SGDAVELSCP PPGGGPMGPT VWVKDGTGLV PSERVLVGPQ RLQVLNASHE DSGAYSCRQR LTQRVLCHFS VRVTDAPSSG DDEDGEDEAE DTGVDTGAPY WTRPERMDKK LLAVPAANTV RFRCPAAGNP TPSISWLKNG REFRGEHRIG GIKLRHQQWS LVMESVVPSD RGNYTCVVEN KFGSIRQTYT LDVLERSPHR PILQAGLPAN QTAVLGSDVE FHCKVYSDAQ PHIQWLKHVE VNGSKVGPDG TPYVTVLKTA GANTTDKELE VLSLHNVTFE DAGEYTCLAG NSIGFSHHSA WLVVLPAEEE LVEADEAGDP RRASIEGRGD PEEPKSCDKT HTCPPCPAPE LLGGPSVFLF PPKPKDTLMI SRTPEVTCVV VDVSHEDPEV KFNWYVDGVE VHNAKTKPRE EQYNSTYRVV SVTVLHQDWL NGKEYKCKVS NKALPAPIEK TISKAKGQPR EPQVYTLPPS RDELTKNQVS LTCLVKGFYP SDIAVEWESN GQPENNYKTT PPVLDSDGSF FLYSKLTVDK SRWQQGNVFS CSVMHEALHN HYTQKSLSLS PGK
purity :
Greater than 90.0% as determined by: (a) Analysis by silver stain. (b) Analysis by SDS-PAGE.
form :
FGFR3 was lyophilized from a concentrated (1 mg/ml) sterile solution containing PBS. Sterile Filtered White lyophilized (freeze-dried) powder.
storage stability :
Lyophilized FGFR3 although stable at room temperature for 3 weeks, should be stored desiccated below -18 degree C. Upon reconstitution FGFR3 should be stored at 4 degree C between 2-7 days and for future use below -18 degree C.For long term storage it is recommended to add a carrier protein (0.1% HSA or BSA).Please prevent freeze-thaw cycles.
other info2 :
Solubility: It is recommended to reconstitute the lyophilized FGFR-3 in sterile PBS not less than 50 ug/ml, which can then be further diluted to other aqueous solutions.
products categories :
PROTEIN KINASES; Enzymes; FGF Receptors
products description :
Description: Soluble FGFR3 Human recombinant fused using a Xa cleavage site with the Fc part of human IgG1 produced in Fc Chimera is a heterodimeric, glycosylated, Polypeptide chain containing 593aa and having a molecular mass of 170kDa. Introduction: Fibroblast Growth Factors (FGFs) comprise a family of at least eighteen structurally realted proteins that are involved in a multitude of physiological and pathological cellular processes, including cell growth, differentation, angiogenesis, wound healing and tumorgenesis. The biological activities of the FGFs are mediated by a family if type I transmembrane tyrosine kinases which undergo dimerization and autophosphorylation after ligand binding. Four distinct genes encoding closely related FGF receptors, FGFR-1to -4 are known. Multiple forms of FGFR-1 to -3 are generated by alternative splicing of the mRNAs. A frequent splicing event involving FGFR-1 and -2 results in receptors containing all three Ig domains, referred to as the alpha isoform, or only IgII and IgIII, referred to as the ? isoform. Only the alpha isoform has been identified for FGFR-3 and FGFR-4. Additional splicing events for FGFR-1 to -3, involving the C-terminal half of the IgIII domain encoded by two mutually exclusive alternative exons, generate FGF receptors with alternative IgIII domains (IIIb and IIIc). A IIIa isoform which is a secreted FGF binding protein containing only the N-terminal half of the IgIII domain plus some intron sequences has also been reported for FGFR-1. Mutations in FGFR-1 to -3 have been found in patients with birth defects involving craniosynostosis.
ncbi acc num :
NP_000133.1
ncbi gb acc num :
NM_000142.4
ncbi mol weight :
85,083 Da
ncbi pathways :
Adaptive Immune System Pathway (366160); Bladder Cancer Pathway (83115); Bladder Cancer Pathway (527); Central Carbon Metabolism In Cancer Pathway (1059538); Central Carbon Metabolism In Cancer Pathway (1084231); Constitutive PI3K/AKT Signaling In Cancer Pathway (685535); DAP12 Interactions Pathway (685549); DAP12 Signaling Pathway (685550); Disease Pathway (530764); Downstream Signal Transduction Pathway (106385)
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. Three alternatively spliced transcript variants that encode different protein isoforms have been described. [provided by RefSeq, Jul 2009]
uniprot summary :
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; Protein kinase, tyrosine (receptor); Membrane protein, integral; Protein kinase, TK; EC 2.7.10.1; TK group; FGFR family. Chromosomal Location of Human Ortholog: 4p16.3. Cellular Component: Golgi apparatus; internal side of plasma membrane; transport vesicle; cell surface; focal adhesion; endoplasmic reticulum; integral to plasma membrane; perinuclear region of cytoplasm; lysosome; extracellular region; plasma membrane; 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; positive regulation of MAPKKK cascade; cell-cell signaling; inner ear receptor cell differentiation; positive regulation of neuron apoptosis; positive regulation of cell proliferation; forebrain development; morphogenesis of an epithelium; chondrocyte differentiation; response to axon injury; skeletal development; negative regulation of epithelial cell proliferation; endochondral ossification; negative regulation of developmental growth; epidermal growth factor receptor signaling pathway; phosphoinositide-mediated signaling; fibroblast growth factor receptor signaling pathway; 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; Crouzon Syndrome With Acanthosis Nigricans; Colorectal Cancer; Lacrimoauriculodentodigital Syndrome; Camptodactyly, Tall Stature, And Hearing Loss Syndrome; Thanatophoric Dysplasia, Type Ii; Testicular Germ Cell Tumor; Nevus, Epidermal