JTK4, CD333, FGFR3, Fibroblast Growth Factor Receptor 3, FGFR-3, Fibroblast Growth Factor Receptor 3 Variant 4, Achondroplasia, Thanatophoric Dwarfism, Hydroxyaryl-Protein Kinase, Tyrosine Kinase JTK4, CD333 Antigen, EC, HSFGFR3EX, CEK2, ACH, Receptor, Fibroblast Growth Factor, Type 3, EC 2.7.10, EC:, Fibroblast Growth Factor Receptor 3-S


Fibroblast growth factor receptor 3 is a protein that in humans is encoded by the FGFR3 gene.FGFR3 has also been designated as CD333 (cluster of differentiation 333). The gene, which is located on chromosome 4, location p16.3, is expressed in tissues such as the cartilage, brain, intestine, and kidneys.The FGFR3 gene produces various forms of the FGFR3 protein; the location varies depending on the isoform of the FGFR3 protein. Since the different forms are found within different tissues the protein is responsible for multiple growth factor interactions.Gain of function mutations in FGFR3 inhibits chondrocyte proliferation and underlies achondroplasia and hypochondroplasia.

KO Status


Drug Information

Launched drugs: 5
Drugs in clinical trials: 15
Latest Research Phase: Approved

Drug Name





Clinical Trials

Lenvatinib Mesylate

ER-203492-00, E-7080


Eisai Co Ltd

Liver Neoplasms, Solid tumours, Biliary Tract Neoplasms, Carcinoma, Renal Cell, Renal Insufficiency, Adenocarcinoma of Lung, Thyroid Carcinoma, Anaplastic, Small Cell Lung Carcinoma, Kidney Diseases, Liver Diseases, Hepatic Insufficiency, Thyroid Neoplasms, Lymphoma, Endometrial Neoplasms, Carcinoma, Hepatocellular, Carcinoma, Non-Small-Cell Lung, Melanoma

Anlotinib Dihydrochloride



Advenchen Laboratories, Jiangsu Chia Tai-Tianqing Pharmaceutical Co Ltd

Biliary Tract Neoplasms, Head and Neck Neoplasms, Solid tumours, Ovarian Neoplasms, Leiomyosarcoma, Liver Neoplasms, Genital Diseases, Female, Stomach Neoplasms, Carcinoma, Renal Cell, Thoracic Neoplasms, Esophageal Neoplasms, Triple Negative Breast Neoplasms, Small Cell Lung Carcinoma, Lymphoma, Large B-Cell, Diffuse, Carcinoma, Ovarian Epithelial, Microsatellite instability-high cancer, Sarcoma, Synovial, Neuroendocrine Tumors, Medullary thyroid cancer (MTC), Sarcoma, Cholangiocarcinoma, Bile Duct Diseases, Nasopharyngeal Carcinoma, Urologic Neoplasms, Colorectal Neoplasms, Gastrointestinal Stromal Tumors, Bone Neoplasms, Peritoneal Neoplasms, Endometrial Neoplasms, Thyroid Neoplasms, Sarcoma, Alveolar Soft Part, Esophageal Squamous Cell Carcinoma, Lung Neoplasms, Fallopian Tube Neoplasms, Gallbladder Neoplasms, Carcinoma, Non-Small-Cell Lung, Uterine Cervical Neoplasms, Carcinoma, Hepatocellular, Gastrointestinal Neoplasms, Melanoma, Adenocarcinoma, Osteoma

Nintedanib Esylate



C.H. Boehringer Sohn Ag & Co. Kg

Ovarian Neoplasms, Solid tumours, Carcinoma, Renal Cell, Scleroderma, Systemic, Idiopathic Pulmonary Fibrosis, Pulmonary Fibrosis, Lung Diseases, Interstitial, Multiple Myeloma, Prostatic Neoplasms, Peritoneal Neoplasms, Hepatic Insufficiency, Genital Neoplasms, Female, Colorectal Neoplasms, Carcinoma, Hepatocellular, Carcinoma, Non-Small-Cell Lung

Pazopanib Hydrochloride

GW-786034, SB-786034, GSK-786034, GW-786034B


Glaxosmithkline Plc, Novartis Pharma Ag

Ovarian Neoplasms, Telangiectasia, Hereditary Hemorrhagic, Chondrosarcoma, Solid tumours, Carcinoma, Renal Cell, Hemangiosarcoma, Small Cell Lung Carcinoma, Pancreatic Neoplasms, Sarcoma, Breast Neoplasms, Osteosarcoma, Prostatic Neoplasms, Colorectal Neoplasms, Peritoneal Neoplasms, Glioma, Fallopian Tube Neoplasms, Carcinoma, Hepatocellular, Melanoma, Carcinoma, Non-Small-Cell Lung, Macular Degeneration


G-024, JNJ-493, 890E37NHMV, JNJ-42756493


Astex Pharmaceuticals Inc

Solid tumours, Carcinoma, Esophageal Neoplasms, Stomach Neoplasms, Carcinoma, Transitional Cell, Urinary Bladder Neoplasms, Bile Duct Neoplasms, Urologic Neoplasms, Hepatic Insufficiency, Lymphoma, Carcinoma, Hepatocellular, Adenocarcinoma, Carcinoma, Non-Small-Cell Lung


BGJ-398, NVP-BGJ398


Novartis Pharma Ag

Solid tumours, Hematologic Neoplasms, Biliary Tract Neoplasms, Esophageal Neoplasms, Stomach Neoplasms, Achondroplasia, Carcinoma, Transitional Cell, Urinary Bladder Neoplasms, Cholangiocarcinoma, Carcinoma, Squamous Cell, Melanoma

Masitinib mesylate



Ab Science

Ovarian Neoplasms, Mastocytosis, Systemic, Pancreatic Neoplasms, Arthritis, Rheumatoid, Coronavirus Disease 2019 (COVID-19), Multiple Myeloma, Multiple Sclerosis, Multiple Sclerosis, Chronic Progressive, Prostatic Neoplasms, Psoriasis, Asthma, Colorectal Neoplasms, Alzheimer Disease, Gastrointestinal Stromal Tumors, Melanoma, Amyotrophic Lateral Sclerosis, Mastocytosis



Phase 3 Clinical

Otsuka Pharmaceutical Co Ltd, Taiho Pharmaceutical Co Ltd

Bone Marrow Neoplasms, Solid tumours, Esophageal Neoplasms, Stomach Neoplasms, Carcinoma, Carcinoma, Transitional Cell, Brain Neoplasms, Cholangiocarcinoma, Breast Neoplasms, Lymphoma


AL-3810, CO-3810, S-80881

Phase 3 Clinical

Advenchen Laboratories Nanjing Ltd

Ovarian Neoplasms, Solid tumours, Stomach Neoplasms, Carcinoma, Transitional Cell, Triple Negative Breast Neoplasms, Small Cell Lung Carcinoma, Breast Neoplasms, Nasopharyngeal Carcinoma, Colorectal Neoplasms, Thymus Neoplasms, Genital Neoplasms, Female, Carcinoma, Small Cell, Lung Neoplasms, Carcinoma, Non-Small-Cell Lung, Carcinoma, Hepatocellular


TA-46, PF-07256472

Phase 2 Clinical

Pfizer Pharmaceuticals Ltd (China)






Asn540Thr substitution in the fibroblast growth factor receptor 3 tyrosine kinase domain causing hypochondroplasia

Deutz-Terlouw P.P., Losekoot M., Aalfs C.M., Hennekam R.C.M., Bakker E.,

Hum. Mutat. Suppl. 1:S62-S65(1998)

Lys650Met substitution in the tyrosine kinase domain of the fibroblast growth factor receptor gene causes thanatophoric dysplasia type I

Kitoh H., Brodie S.G., Kupke K.G., Lachman R.S., Wilcox W.R.,

Hum. Mutat. 12:362-363(1998)

Deafness due to Pro250Arg mutation of FGFR3

Hollway G.E., Suthers G.K., Battese K.M., Turner A.M., David D.J., Mulley J.C.,

Lancet 351:877-878(1998)

Platyspondylic lethal skeletal dysplasia, San Diego type, is caused by FGFR3 mutations

Brodie S.G., Kitoh H., Lachman R.S., Nolasco L.M., Mekikian P.B., Wilcox W.R.,

Am. J. Med. Genet. 84:476-480(1999)

Sex related expressivity of the phenotype in coronal craniosynostosis caused by the recurrent P250R FGFR3 mutation

Lajeunie E., El Ghouzzi V., Le Merrer M., Munnich A., Bonaventure J., Renier D.,

J. Med. Genet. 36:9-13(1999)

Frequent activating mutations of FGFR3 in human bladder and cervix carcinomas

Cappellen D., De Oliveira C., Ricol D., Gil Diez de Medina S., Bourdin J., Sastre-Garau X., Chopin D., Thiery J.P., Radvanyi F.,

Nat. Genet. 23:18-20(1999)

Distinct missense mutations of the FGFR3 Lys650 codon modulate receptor kinase activation and the severity of the skeletal dysplasia phenotype

Bellus G.A., Spector E.B., Speiser P.W., Weaver C.A., Garber A.T., Bryke C.R., Israel J., Rosengren S.S., Webster M.K., Donoghue D.J., Francomano C.A.,

Am. J. Hum. Genet. 67:1411-1421(2000)

Clinical and radiographic features of a family with hypochondroplasia owing to a novel asn540ser mutation in the fibroblast growth factor receptor 3 gene

Mortier G., Nuytinck L., Craen M., Renard J.-P., Leroy J.G., De Paepe A.,

J. Med. Genet. 37:220-224(2000)

Syndrome of coronal craniosynostosis, Klippel-Feil anomaly, and sprengel shoulder with and without Pro250Arg mutation in the FGFR3 gene

Lowry R.B., Jabs E.W., Graham G.E., Gerritsen J., Fleming J.,

Am. J. Med. Genet. 104:112-119(2001)

Analysis of FGFR3 gene mutations in multiple myeloma patients with t(4;14)

Intini D., Baldini L., Fabris S., Lombardi L., Ciceri G., Maiolo A.T., Neri A.,

Br. J. Haematol. 114:362-364(2001)