Fibroblast Growth Factor Receptor 3 (FGFR3) Active Protein | FGFR3 active protein

Recombinant Human Fibroblast Growth Factor Receptor 3 (FGFR3), Partial

Cat. Num. AAA7114984

• Gene Names: FGFR3; ACH; CEK2; JTK4; CD333; HSFGFR3EX
• Purity: Greater or equal to 85% purity as determined by SDS-PAGE.
• Synonyms: Fibroblast Growth Factor Receptor 3 (FGFR3); Recombinant Human Fibroblast Growth Factor Receptor 3 (FGFR3); Partial; Fibroblast growth factor receptor 3; FGFR-3; CD333; FGFR3; JTK4; IIIc; FGFR3 active protein

• Host: Mammalian Cell
• 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.
• Purity/Purification: Greater or equal to 85% purity as determined by SDS-PAGE.
• Form/Format: Lyophilized from a 0.2 mum Filtered 1xPBS, pH 7.4
• Sequence Positions: 23-375aa; Partial
• Sequence: ESLGTEQRVVGRAAEVPGPEPGQQEQLVFGSGDAVELSCPPPGGGPMGPTVWVKDGTGLVPSERVLVGPQRLQVLNASHEDSGAYSCRQRLTQRVLCHFSVRVTDAPSSGDDEDGEDEAEDTGVDTGAPYWTRPERMDKKLLAVPAANTVRFRCPAAGNPTPSISWLKNGREFRGEHRIGGIKLRHQQWSLVMESVVPSDRGNYTCVVENKFGSIRQTYTLDVLERSPHRPILQAGLPANQTAVLGSDVEFHCKVYSDAQPHIQWLKHVEVNGSKVGPDGTPYVTVLKTAGANTTDKELEVLSLHNVTFEDAGEYTCLAGNSIGFSHHSAWLVVLPAEEELVEADEAGSVYAG
• Sequence Length: 806

• Species: Human
• Tag: C-terminal FC-tagged
• Endotoxin: Less than 1.0 EU/ug as determined by LAL method.
• 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.
• 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
• Classification: Cytokine
• Subdivision: Growth Factor
• Pathway: MAPK Signaling Pathway
• Preparation and Storage: Store at -20 degree C. For extended storage, conserve at -20 degree C or -80 degree C. Repeated freezing and thawing is not recommended. Store working aliquots at 4 degree C for up to one week.

Related Product Information for FGFR3 active protein

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 alpha isoform, or only IgII and IgIII, referred to as the beta isoform. Only the alpha 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.

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.

Product Categories/Family for FGFR3 active protein

Signal Transduction

NCBI and Uniprot Product Information

• NCBI GI #: 4503711
• NCBI GeneID: 2261
• NCBI Accession #: NP_000133.1
• NCBI GenBank Nucleotide #: NM_000142.4
• UniProt Accession #: P22607
• Molecular Weight: 64.8 kDa
• NCBI Official Full Name: fibroblast growth factor receptor 3 isoform 1
• NCBI Official Synonym Full Names: fibroblast growth factor receptor 3
• NCBI Official Symbol: FGFR3
• NCBI Official Synonym Symbols: ACH; CEK2; JTK4; CD333; HSFGFR3EX
• NCBI Protein Information: fibroblast growth factor receptor 3
• UniProt Protein Name: Fibroblast growth factor receptor 3
• Protein Family: Fibroblast growth factor receptor
• UniProt Gene Name: FGFR3
• UniProt Synonym Gene Names: JTK4; FGFR-3
• UniProt Entry Name: FGFR3_HUMAN

NCBI Description

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 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. 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: Protein kinase, tyrosine (receptor); Protein kinase, TK; Kinase, protein; Membrane protein, integral; 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; focal adhesion; cell surface; perinuclear region of cytoplasm; integral to plasma membrane; endoplasmic reticulum; 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; inner ear receptor cell differentiation; positive regulation of MAPKKK cascade; cell-cell signaling; positive regulation of neuron apoptosis; forebrain development; positive regulation of cell proliferation; morphogenesis of an epithelium; chondrocyte differentiation; 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; 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; positive regulation of endothelial cell proliferation; lens morphogenesis in camera-type eye; 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