Dysregulated PDGFR? signaling alters coronal suture morphogenesis and leads to craniosynostosis through endochondral ossification.
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ABSTRACT:
SUBMITTER: He F
PROVIDER: S-EPMC5702073 | biostudies-literature | 2017 Nov
REPOSITORIES: biostudies-literature
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Dysregulated PDGFRα signaling alters coronal suture morphogenesis and leads to craniosynostosis through endochondral ossification.
He Fenglei F Soriano Philippe P
Development (Cambridge, England) 20170925 21
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 ...[more]
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