Dysregulated PDGFRα signaling alters coronal suture morphogenesis and leads to craniosynostosis through endochondral ossification

Fenglei He, Philippe Soriano

Research output: Contribution to journalArticlepeer-review

13 Scopus citations

Abstract

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 dysregulated 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.

Original languageEnglish
Pages (from-to)4026-4036
Number of pages11
JournalDevelopment (Cambridge)
Volume144
Issue number21
DOIs
StatePublished - 1 Nov 2017

Keywords

  • Craniosynostosis
  • Endochondral ossification
  • Mesoderm
  • Neural crest
  • Receptor tyrosine kinase

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