Open AccessDeregulated PDGFRα signaling alters coronal suture morphogenesis and leads to craniosynostosis through endochondral ossification
Author(s) -
Fenglei He,
Philippe Soriano
Publication year - 2017
Publication title -
development
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.15
H-Index - 36
eISSN - 1477-9129
pISSN - 0950-1991
DOI - 10.1242/dev.151068
Subject(s) - craniosynostosis , biology , endochondral ossification , ossification , coronal suture , fibrous joint , microbiology and biotechnology , intramembranous ossification , anatomy , cartilage
Craniosynostosis is a prevalent human birth defect characterized by premature fusion of calvarial bones. In this study, we show that tight regulation of endogenous PDGFRα activity is required for normal calvarium development in the mouse and that deregulated PDGFRα activity causes craniosynostosis. Constitutive activation of PDGFRα leads to expansion of cartilage underlying the coronal sutures, which contribute to suture closure through endochondral ossification, in a process regulated in part by PI3K/Akt signaling. Our results thus identify a novel mechanism underlying calvarial development in craniosynostosis.