biostudies-literatureNita AAgency for Healthcare Research of the Czech RepublicNational Institute for Cancer ResearchIGA MUNIMinistry of Education, Youth and Sports of the Czech RepublicEuropean UnionAgency of the Masaryk UniversityBrno City MunicipalityCzech Science Foundatione202311030https://www.ebi.ac.uk/biostudies/studies/S-EPMC12010920biostudies-literatureUnknown224(7)Primary cilium projects from cells to provide a communication platform with neighboring cells and the surrounding environment. This is ensured by the selective entry of membrane receptors and signaling molecules, producing fine-tuned and effective responses to the extracellular cues. In this study, we focused on one family of signaling molecules, the fibroblast growth factor receptors (FGFRs), their residence within cilia, and its role in FGFR signaling. We show that FGFR1 and FGFR2, but not FGFR3 and FGFR4, localize to primary cilia of the developing mouse tissues and in vitro cells. For FGFR2, we demonstrate that the ciliary residence is necessary for its signaling and expression of target morphogenic genes. We also show that the pathogenic FGFR2 variants have minimal cilium presence, whThe Journal of cell biologyFGFR2 residence in primary cilia is necessary for epithelial cell signaling.PMC12010920NU21-06-00512GA23-07631SLX22NPO5102LUAUS23295MUNI/G/1771/2020NW24-08-00364NU22-07-00380CZ.02.2.69/0.0/0.0/19_073/0016943GF21-26400KElrefaay ERTrantirek LAbraham SPFafilek BGudernova IRico GBarta TUrsachi VCCizkova EFeketova ZWiedlocha AGregor TKoudelka ADudeja PCzyrek AASvozilova KCeliker CNita AKrejci PBosakova MfalseFGFR2 residence in primary cilia is necessary for epithelial cell signaling.Primary cilium projects from cells to provide a communication platform with neighboring cells and the surrounding environment. This is ensured by the selective entry of membrane receptors and signaling molecules, producing fine-tuned and effective responses to the extracellular cues. In this study, we focused on one family of signaling molecules, the fibroblast growth factor receptors (FGFRs), their residence within cilia, and its role in FGFR signaling. We show that FGFR1 and FGFR2, but not FGFR3 and FGFR4, localize to primary cilia of the developing mouse tissues and in vitro cells. For FGFR2, we demonstrate that the ciliary residence is necessary for its signaling and expression of target morphogenic genes. We also show that the pathogenic FGFR2 variants have minimal cilium presence, wh2025-01-01T00:00:00Z2025 Jul2026-06-01T12:53:59.459Z2025-07-01T03:05:37.616ZS-EPMC120109204025737810.1083/jcb.202311030