{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,20]],"date-time":"2026-09-20T09:42:17Z","timestamp":1789897337996,"version":"4.0.1"},"reference-count":27,"publisher":"Wiley","issue":"4","license":[{"start":{"date-parts":[[2013,12,19]],"date-time":"2013-12-19T00:00:00Z","timestamp":1387411200000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc-nd\/3.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Developmental Dynamics"],"published-print":{"date-parts":[[2014,4]]},"abstract":"<jats:p><jats:styled-content>Background:<\/jats:styled-content> Apert syndrome (AS) is characterized by craniosynostosis, midfacial hypoplasia, and bony syndactyly. It is an autosomal dominantly inherited disease caused by point mutations (S252W or P253R) in fibroblast growth factor receptor (FGFR) 2. These mutations cause activation of FGFR2 depending on ligand binding. Recently, an AS mouse model, <jats:italic>Fgfr2<jats:sup>+\/<\/jats:sup><jats:sup>S252W<\/jats:sup><\/jats:italic>, showed phenotypes similar to those of AS patients. We previously reported that the soluble form of FGFR2<jats:sup>S252W<\/jats:sup> (sFGFR2IIIc<jats:sup>S252W<\/jats:sup>) efficiently inhibits enhanced osteoblastic differentiation caused by FGFR2 activation in AS in vitro, presumably because FGFs binding to FGFRs is interrupted. In this study, we developed <jats:italic>Fgfr2<jats:sup>+\/<\/jats:sup><jats:sup>S252W<\/jats:sup><\/jats:italic> (Ap) mice expressing the sFGFR2IIIc<jats:sup>S252W<\/jats:sup> protein, and we investigated the effects of sFGFR2IIIc<jats:sup>S252W<\/jats:sup> on AS\u2010like phenotypes. <jats:styled-content>Results:<\/jats:styled-content> In Ap mice, the coronal suture (CS) was fused prematurely at P1. In addition, the mice exhibited a widened interfrontal suture (IFS) with ectopic bone and thickened cartilage formation. In <jats:italic>Fgfr2<jats:sup>+\/<\/jats:sup><jats:sup>S252W<\/jats:sup> sFGFR2IIIc<jats:sup>S252W<\/jats:sup><\/jats:italic> (Ap\/Sol) mice, the CS was similar to that of wild\u2010type mice. Ap\/Sol mice did not show any ectopic bone or cartilage formation in the IFS, but showed a wider IFS than that of the wild\u2010type mice. <jats:styled-content>Conclusions:<\/jats:styled-content> sFGFR2IIIc<jats:sup>S252W<\/jats:sup> may partially prevent craniosynostosis in the Apert mouse model by affecting the CS and IFS in vivo. <jats:italic>Developmental Dynamics 243:560\u2013567, 2014<\/jats:italic>. \u00a9 2013 The Authors Developmental Dynamics published by Wiley Periodicals, Inc. on behalf of American Association of Anatomists.<\/jats:p>","DOI":"10.1002\/dvdy.24099","type":"journal-article","created":{"date-parts":[[2013,11,21]],"date-time":"2013-11-21T01:30:29Z","timestamp":1384997429000},"page":"560-567","source":"Crossref","is-referenced-by-count":29,"title":["Soluble form of FGFR2 with S252W partially prevents craniosynostosis of the apert mouse model"],"prefix":"10.1002","volume":"243","author":[{"given":"Jumpei","family":"Morita","sequence":"first","affiliation":[{"name":"Maxillofacial Orthognathics, Department of Maxillofacial Reconstruction and Function Graduate School of Medical and Dental Sciences Tokyo Medical and Dental University Tokyo Japan"},{"name":"Human Gene Sciences Center Tokyo Medical and Dental University Tokyo Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Masataka","family":"Nakamura","sequence":"additional","affiliation":[{"name":"Human Gene Sciences Center Tokyo Medical and Dental University Tokyo Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yukiho","family":"Kobayashi","sequence":"additional","affiliation":[{"name":"Maxillofacial Orthognathics, Department of Maxillofacial Reconstruction and Function Graduate School of Medical and Dental Sciences Tokyo Medical and Dental University Tokyo Japan"},{"name":"Hard Tissue Genome Research Center Tokyo Medical and Dental University Tokyo Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Chu\u2010Xia","family":"Deng","sequence":"additional","affiliation":[{"name":"Genetics of Development and Disease Branch National Institute of Diabetes, Digestive and Kidney Diseases, US National Institutes of Health Bethesda Maryland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Noriko","family":"Funato","sequence":"additional","affiliation":[{"name":"Human Gene Sciences Center Tokyo Medical and Dental University Tokyo Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Keiji","family":"Moriyama","sequence":"additional","affiliation":[{"name":"Maxillofacial Orthognathics, Department of Maxillofacial Reconstruction and Function Graduate School of Medical and Dental Sciences Tokyo Medical and Dental University Tokyo Japan"},{"name":"Hard Tissue Genome Research Center Tokyo Medical and Dental University Tokyo 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