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

First Author  He F Year  2017
Journal  Development Volume  144
Issue  21 Pages  4026-4036
PubMed ID  28947535 Mgi Jnum  J:245766
Mgi Id  MGI:5915646 Doi  10.1242/dev.151068
Citation  He F, et al. (2017) Dysregulated PDGFRalpha signaling alters coronal suture morphogenesis and leads to craniosynostosis through endochondral ossification. Development 144(21):4026-4036
abstractText  Craniosynostosis is a prevalent human birth defect characterized by premature fusion of calvarial bones. In this study, we show that tight regulation of endogenous PDGFRalpha activity is required for normal calvarium development in the mouse and that dysregulated PDGFRalpha activity causes craniosynostosis. Constitutive activation of PDGFRalpha 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.
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