FGFR3 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. Belongs to the protein kinase superfamily. Tyr protein kinase family. Fibroblast growth factor receptor subfamily. 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. 4 alternatively spliced human isoforms have been reported. Note: This description may include information from UniProtKB.
Protein type: EC; FGFR family; Kinase, protein; Membrane protein, integral; Protein kinase, TK; Protein kinase, tyrosine (receptor); TK group
Chromosomal Location of human Ortholog: 4p16.3
Cellular Component:  cell surface; endoplasmic reticulum; extracellular region; Golgi apparatus; integral component of plasma membrane; nucleus; plasma membrane; transport vesicle
Molecular Function:  ATP binding; fibroblast growth factor binding; fibroblast growth factor-activated receptor activity; identical protein binding; protein binding; protein tyrosine kinase activity
Biological Process:  bone maturation; bone mineralization; bone morphogenesis; cell-cell signaling; chondrocyte differentiation; chondrocyte proliferation; endochondral bone growth; endochondral ossification; fibroblast growth factor receptor apoptotic signaling pathway; fibroblast growth factor receptor signaling pathway; MAPK cascade; negative regulation of developmental growth; peptidyl-tyrosine phosphorylation; positive regulation of cell proliferation; positive regulation of ERK1 and ERK2 cascade; positive regulation of MAPK cascade; positive regulation of phosphatidylinositol 3-kinase activity; positive regulation of phospholipase activity; positive regulation of protein kinase B signaling; positive regulation of tyrosine phosphorylation of STAT protein; protein autophosphorylation; skeletal system development
Disease: Achondroplasia; Achondroplasia, Severe, With Developmental Delay And Acanthosis Nigricans; Bladder Cancer; Camptodactyly, Tall Stature, And Hearing Loss Syndrome; Cervical Cancer; Colorectal Cancer; Crouzon Syndrome With Acanthosis Nigricans; Hypochondroplasia; Lacrimoauriculodentodigital Syndrome; Muenke Syndrome; Nevus, Epidermal; Testicular Germ Cell Tumor; Thanatophoric Dysplasia, Type I; Thanatophoric Dysplasia, Type Ii
Reference #:  P22607 (UniProtKB)
Alt. Names/Synonyms: ACH; achondroplasia, thanatophoric dwarfism; CD333; CEK2; FGFR-3; FGFR3; Fibroblast growth factor receptor 3; fibroblast growth factor receptor 3 variant 4; fibroblast growth factor receptor 3-S; HSFGFR3EX; hydroxyaryl-protein kinase; JTK4; tyrosine kinase JTK4
Gene Symbols: FGFR3
Molecular weight: 87,710 Da
Basal Isoelectric point: 5.59  Predict pI for various phosphorylation states
CST Pathways:  Angiogenesis  |  ESC Pluripotency and Differentiation  |  Tyrosine Kinases & Substrates
Protein-Specific Antibodies, siRNAs or Recombinant Proteins from Cell Signaling Technology® Total Proteins
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Protein Structure Not Found.

Cross-references to other databases:  AlphaFold  |  STRING  |  cBioPortal  |  Wikipedia  |  Reactome  |  neXtProt  |  Protein Atlas  |  BioGPS  |  KinBase  |  Pfam  |  RCSB PDB  |  ENZYME  |  Phospho3D  |  Phospho.ELM  |  NetworKIN  |  GeneCards  |  UniProtKB  |  Entrez-Gene  |  GenPept  |  Ensembl Gene  |  InnateDB  |  Ensembl Protein