Tags

Type your tag names separated by a space and hit enter

Dysregulated PDGFRα signaling alters coronal suture morphogenesis and leads to craniosynostosis through endochondral ossification.
Development 2017; 144(21):4026-4036D

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.

Authors+Show Affiliations

Department of Cell, Developmental and Regenerative Biology, Icahn School of Medicine at Mount Sinai, New York, NY 10029, USA. Department of Cell and Molecular Biology, Tulane University, New Orleans, LA 70118, USA.Department of Cell, Developmental and Regenerative Biology, Icahn School of Medicine at Mount Sinai, New York, NY 10029, USA philippe.soriano@mssm.edu.

Pub Type(s)

Journal Article

Language

eng

PubMed ID

28947535

Citation

He, Fenglei, and Philippe Soriano. "Dysregulated PDGFRα Signaling Alters Coronal Suture Morphogenesis and Leads to Craniosynostosis Through Endochondral Ossification." Development (Cambridge, England), vol. 144, no. 21, 2017, pp. 4026-4036.
He F, Soriano P. Dysregulated PDGFRα signaling alters coronal suture morphogenesis and leads to craniosynostosis through endochondral ossification. Development. 2017;144(21):4026-4036.
He, F., & Soriano, P. (2017). Dysregulated PDGFRα signaling alters coronal suture morphogenesis and leads to craniosynostosis through endochondral ossification. Development (Cambridge, England), 144(21), pp. 4026-4036. doi:10.1242/dev.151068.
He F, Soriano P. Dysregulated PDGFRα Signaling Alters Coronal Suture Morphogenesis and Leads to Craniosynostosis Through Endochondral Ossification. Development. 2017 11 1;144(21):4026-4036. PubMed PMID: 28947535.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Dysregulated PDGFRα signaling alters coronal suture morphogenesis and leads to craniosynostosis through endochondral ossification. AU - He,Fenglei, AU - Soriano,Philippe, Y1 - 2017/09/25/ PY - 2017/02/23/received PY - 2017/09/15/accepted PY - 2017/9/28/pubmed PY - 2017/11/29/medline PY - 2017/9/27/entrez KW - Craniosynostosis KW - Endochondral ossification KW - Mesoderm KW - Neural crest KW - Receptor tyrosine kinase SP - 4026 EP - 4036 JF - Development (Cambridge, England) JO - Development VL - 144 IS - 21 N2 - 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. SN - 1477-9129 UR - https://www.unboundmedicine.com/medline/citation/28947535/Dysregulated_PDGFRα_signaling_alters_coronal_suture_morphogenesis_and_leads_to_craniosynostosis_through_endochondral_ossification_ L2 - http://dev.biologists.org/cgi/pmidlookup?view=long&pmid=28947535 DB - PRIME DP - Unbound Medicine ER -