FGFR2

Chr 10

fibroblast growth factor receptor 2

Also known as: BBDS, BEK, BFR-1, CD332, CEK3, CFD1, ECT1, JWS

The protein encoded by this gene is a member of the fibroblast growth factor receptor family, where amino acid sequence is highly conserved between members and throughout evolution. FGFR family members differ from one another in their ligand affinities and tissue distribution. A full-length representative protein consists 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 is a high-affinity receptor for acidic, basic and/or keratinocyte growth factor, depending on the isoform. Mutations in this gene are associated with Crouzon syndrome, Pfeiffer syndrome, Craniosynostosis, Apert syndrome, Jackson-Weiss syndrome, Beare-Stevenson cutis gyrata syndrome, Saethre-Chotzen syndrome, and syndromic craniosynostosis. Multiple alternatively spliced transcript variants encoding different isoforms have been noted for this gene. [provided by RefSeq, Jan 2009]

GeneReviewsResearchGenerating clinical summary…

Primary Disease Associations & Inheritance

UniProtCrouzon syndrome
UniProtJackson-Weiss syndrome
UniProtApert syndrome
UniProtPfeiffer syndrome

Clinical highlights

Gene-disease validity (ClinGen)
Apert syndrome · ADDefinitivesufficient evidence for diagnostic panels6 gene-disease associations curated in total
Interpreting a novel variant
Gain of function is the curated mechanism (Gene2Phenotype), so a variant that simply removes the protein may not be the pathogenic class here — missense variants in functional domains often carry more weight.Curated gene-level mechanism — a prior for triage, not a per-variant call.
12
Active trials
561
Pubs (1 yr)
P/LP submissions
P/LP missense
0.27
LOEUF· LoF intol.
GOF*
Mechanism· G2P
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GeneReview available — FGFR2
Authoritative clinical overview · Recommended first read
Open GeneReview ↗
Some data sources returned errors (1)

omim: Error: OMIM fetch failed: 429

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

LoF intolerant — likely haploinsufficient
LoF Constraint?
0.27LOEUF
pLI 0.997
Z-score 5.29
OE 0.14 (0.070.27)
Highly constrained

Highly LoF-intolerant (top ~10% of genes)

Missense Constraint?
2.40Z-score
OE missense 0.69 (0.630.75)
323 obs / 469.5 exp
Mild constraint

Moderately missense-constrained (top ~2.5%)

Observed / Expected Ratios?
LoF OE?0.14 (0.070.27)
00.351.4
Missense OE?0.69 (0.630.75)
00.61.4
Synonymous OE?1.11
01.21.6
LoF obs/exp: 6 / 43.8Missense obs/exp: 323 / 469.5Syn Z: -1.17

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

FGFR2 · protein map & ClinVar variants

Showing all ClinVar variants across the protein. Search a specific variant to highlight its position.

Clinical Trials

Active and recruiting trials from ClinicalTrials.gov

Locally Advanced Bladder Urothelial CarcinomaLocally Advanced Renal Pelvis Urothelial CarcinomaLocally Advanced Ureter Urothelial Carcinoma

Testing Combination Erdafitinib and Enfortumab Vedotin in Metastatic Bladder Cancer After Treatment With Chemotherapy and Immunotherapy

ACTIVE NOT RECRUITING
NCT04963153Phase PHASE1National Cancer Institute (NCI)Started 2022-07-07
Biospecimen CollectionBone ScanComputed Tomography
Cholangiocarcinoma,Adult

Efficacy and Safety of 3D185 Monotherapy in Subjects With Previously Treated Locally Advanced or Metastatic Cholangiocarcinoma

NOT YET RECRUITING
NCT05039892Phase PHASE23D Medicines (Beijing) Co., Ltd.Started 2026-12
3D185
Rare Malignant Neoplasm

Marker Assisted Selective ThErapy in Rare Cancers: Knowledge Database Establishing registrY Asia

RECRUITING
NCT05217407National Cancer Center, JapanStarted 2021-11-30
Genomic sequence
HER2-Gastric/Gastroesophageal Junction CancerUrothelial CarcinomaNon-Small Cell Lung Cancer

A Phase 2 Clinical Study of ABSK061 and ABSK043

RECRUITING
NCT06632262Phase PHASE2Abbisko Therapeutics Co, LtdStarted 2024-11-13
ABSK061 + ABSK043ABSK061+ABSK043 in combination with CAPOX
Intrahepatic Cholangiocarcinoma (Icc)Advanced Cholangiocarcinoma

Efficacy and Safety of TT-00420 (Tinengotinib) Tablets Versus Chemotherapy in Patients With Advanced Intrahepatic Cholangiocarcinoma Harboring FGFR2 Fusions/Rearrangements or Mutations

NOT YET RECRUITING
NCT07328919Phase PHASE3TransThera Sciences (Nanjing), Inc.Started 2026-01
TT-00420 (tinengotinib)Oxaliplatin, fluorouracil, calcium folinate, irinotecan, capecitabine
Intrahepatic CholangiocarcinomaFGFR2 Gene RearrangementFGFR2 Gene Mutation

A Phase II Study of Pemigatinib Plus Durvalumab in Previously Treated Advanced Intrahepatic Cholangiocarcinoma Patients With FGFR-2 Fusion or Rearrangement

RECRUITING
NCT06728410Phase PHASE2Mehmet AkceStarted 2026-01-27
PemigatinibDurvalumab
Locally Advanced CholangiocarcinomaIntrahepatic CholangiocarcinomaSolid Tumor

Safety and Anti-Tumor Activity of TYRA-200 in Advanced Cholangiocarcinoma With Activating FGFR2 Gene Alterations

ACTIVE NOT RECRUITING
NCT06160752Phase PHASE1Tyra Biosciences, IncStarted 2023-11-22
Phase 1 Part A - dose escalation TYRA-200 taken once daily by mouth in 28-day cyclesPhase 1 Part B - dose expansion TYRA-200 taken once daily by mouth in 28-day cycles
Lymphoma, Non-HodgkinMultiple MyelomaAdvanced Solid Tumors

Canadian Profiling and Targeted Agent Utilization Trial (CAPTUR)

RECRUITING
NCT03297606Phase PHASE2Canadian Cancer Trials GroupStarted 2018-03-23
OlaparibDasatinibNivolumab plus Ipilimumab
Advanced Malignant Solid NeoplasmAnn Arbor Stage III Non-Hodgkin LymphomaAnn Arbor Stage IV Non-Hodgkin Lymphoma

Targeted Therapy Directed by Genetic Testing in Treating Pediatric Patients With Relapsed or Refractory Advanced Solid Tumors, Non-Hodgkin Lymphomas, or Histiocytic Disorders (The Pediatric MATCH Screening Trial)

ACTIVE NOT RECRUITING
NCT03155620Phase PHASE2National Cancer Institute (NCI)Started 2017-07-31
Biopsy ProcedureBiospecimen CollectionBone Marrow Aspiration and Biopsy
Gastric and Cardia Adenocarcinomas

Biomoleculars Markers of Sensitivity to Pre- and Post-operative Chemotherapy of Gastric and Cardia Adenocarcinomas: a Pilot Study

RECRUITING
NCT02491840Phase NAUniversity Hospital, Strasbourg, FranceStarted 2016-05
Biopsy
Biliary Tract Cancer

A Post-marketing Observational Study of Tasfygo in Participants With Unresectable Biliary Tract Cancer With Fibroblast Growth Factor Receptor 2 (FGFR2) Fusion Gene Positivity Who Progressed After Chemotherapy

RECRUITING
NCT06793709Eisai Co., Ltd.Started 2025-07-10
No Intervention
Intrahepatic Cholangiocarcinoma (Icc)CholangiocarcinomaOther Solid Tumors, Adult

A Study of an FGFR2/3 Inhibitor (CGT4859) in Patients With Cholangiocarcinoma and Other Advanced Solid Tumors

ACTIVE NOT RECRUITING
NCT06777316Phase PHASE1, PHASE2Cogent Biosciences, Inc.Started 2025-01-22
CGT4859