Recombinant Human Fibroblast growth factor receptor 3 (FGFR3), partial (Active) | CSB-AP003871HU

Recombinant Human Fibroblast growth factor receptor 3 (FGFR3) ,partial (Active) | CSB-AP003871HU | Cusabio

Protein Description: Partial

Alternative Name (s) : Fibroblast growth factor receptor 3; FGFR-3; CD333; FGFR3; JTK4; IIIc

Gene Names: FGFR3

Research Areas: Signal Transduction

Species: Homo sapiens (Human)

Source: Mammalian cell

Tag Info: C-terminal Fc-tagged

Expression Region: 23-375aa

Sequence Info: ESLGTEQRVVGRAAEVPGPEPGQQEQLVFGSGDAVELSCPPPGGGPMGPTVWVKDGTGLVPSERVLVGPQRLQVLNASHEDSGAYSCRQRLTQRVLCHFSVRVTDAPSSGDDEDGEDEAEDTGVDTGAPYWTRPERMDKKLLAVPAANTVRFRCPAAGNPTPSISWLKNGREFRGEHRIGGIKLRHQQWSLVMESVVPSDRGNYTCVVENKFGSIRQTYTLDVLERSPHRPILQAGLPANQTAVLGSDVEFHCKVYSDAQPHIQWLKHVEVNGSKVGPDGTPYVTVLKTAGANTTDKELEVLSLHNVTFEDAGEYTCLAGNSIGFSHHSAWLVVLPAEEELVEADEAGSVYAG

Biological Activity: The ED50 as determined by its ability to inhibit FGF acidic-dependent proliferation of BALB/c 3T3 cells is less than 100 ng/ml.

MW: 64.8 kDa

Purity: Greater than 95% as determined by SDS-PAGE.

Endotoxin: Less than 1.0 EU/µg as determined by LAL method.

Relevance: Fibroblast growth factors (FGFs) are involved in a multitude of physiological and pathological cellular processes. The biological activities of the FGFs are mediated by a family of type I transmembrane tyrosine kinases which undergo dimerization and autophosphorylation after ligand binding. Four distinct genes encoding closely related FGF receptors, FGF R1-4, are known. All four genes for FGF Rs encode proteins with an N-terminal signal peptide, three immunoglobulin (Ig) -like domains, an acid-box region containing a run of acidic residues between the IgI and IgII domains, a transmembrane domain and the split tyrosine-kinase domain. Multiple forms of FGF R1-3 are generated by alternative splicing of the mRNAs. A frequent splicing event involving FGF R1 and 2 results in receptors containing all three Ig domains, referred to as the α isoform, or only IgII and IgIII, referred to as the β isoform. Only the α isoform has been identified for FGF R3 and FGF R4. Additional splicing events for FGF R1-3, 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) . The complex patterns of expression of these receptors as well as the specificity of their interactions with the various FGF ligand family members are under investigation.

PubMed ID:

Notes: Repeated freezing and thawing is not recommended. Store working aliquots at 4℃ for up to one week.

Function: 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.

Involvement in disease: Achondroplasia (ACH) ; Crouzon syndrome with acanthosis nigricans (CAN) ; Thanatophoric dysplasia 1 (TD1) ; Thanatophoric dysplasia 2 (TD2) ; Hypochondroplasia (HCH) ; Bladder cancer (BLC) ; Cervical cancer (CERCA) ; Camptodactyly, tall stature, and hearing loss syndrome (CATSHLS) ; Multiple myeloma (MM) ; Lacrimo-auriculo-dento-digital syndrome (LADDS) ; Keratinocytic non-epidermolytic nevus (KNEN) ; Muenke syndrome (MNKS) ; Keratosis, seborrheic (KERSEB) ; Testicular germ cell tumor (TGCT) ; Achondroplasia, severe, with developmental delay and acanthosis nigricans (SADDAN)

Subcellular Location: Isoform 1: Cell membrane, Single-pass type I membrane protein, Cytoplasmic vesicle, Endoplasmic reticulum

Protein Families: Protein kinase superfamily, Tyr protein kinase family, Fibroblast growth factor receptor subfamily

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.

Paythway: MAPKsignalingpathway

Form: Lyophilized powder

Buffer: Lyophilized from a 0.2 μm filtered 1xPBS, pH 7.4

Reconstitution: We recommend that this vial be briefly centrifuged prior to opening to bring the contents to the bottom. Please reconstitute protein in deionized sterile water to a concentration of 0.1-1.0 mg/mL.We recommend to add 5-50% of glycerol (final concentration) and aliquot for long-term storage at -20℃/-80℃. Our default final concentration of glycerol is 50%. Customers could use it as reference.

Uniprot ID: P22607

Uniprot Entry Name:

HGNC Database Link: HGNC

UniGene Database Link: UniGene

KEGG Database Link: KEGG

STRING Database Link: STRING

OMIM Database Link: OMIM