Tags

Type your tag names separated by a space and hit enter

Differential activation of canonical Wnt signaling determines cranial sutures fate: a novel mechanism for sagittal suture craniosynostosis.
Dev Biol 2010; 344(2):922-40DB

Abstract

Premature closure of cranial sutures, which serve as growth centers for the skull vault, result in craniosynostosis. In the mouse posterior frontal (PF) suture closes by endochondral ossification, whereas sagittal (SAG) remain patent life time, although both are neural crest tissue derived. We therefore, investigated why cranial sutures of same tissue origin adopt a different fate. We demonstrated that closure of the PF suture is tightly regulated by canonical Wnt signaling, whereas patency of the SAG suture is achieved by constantly activated canonical Wnt signaling. Importantly, the fate of PF and SAG sutures can be reversed by manipulating Wnt signaling. Continuous activation of canonical Wnt signaling in the PF suture inhibits endochondral ossification and therefore, suture closure, In contrast, inhibition of canonical Wnt signaling in the SAG suture, upon treatment with Wnt antagonists results in endochondral ossification and suture closure. Thus, inhibition of canonical Wnt signaling in the SAG suture phenocopies craniosynostosis. Moreover, mice haploinsufficient for Twist1, a target gene of canonical Wnt signaling which inhibits chondrogenesis, have sagittal craniosynostosis. We propose that regulation of canonical Wnt signaling is of crucial importance during the physiological patterning of PF and SAG sutures. Importantly, dysregulation of this pathway may lead to craniosynostosis.

Authors+Show Affiliations

Children's Surgical Research Program, Department of Surgery, Division of Plastic and Reconstructive Surgery, Stanford University School of Medicine, Stanford, CA, USA.No affiliation info availableNo affiliation info available

Pub Type(s)

Journal Article
Research Support, N.I.H., Extramural
Research Support, Non-U.S. Gov't

Language

eng

PubMed ID

20547147

Citation

Behr, Björn, et al. "Differential Activation of Canonical Wnt Signaling Determines Cranial Sutures Fate: a Novel Mechanism for Sagittal Suture Craniosynostosis." Developmental Biology, vol. 344, no. 2, 2010, pp. 922-40.
Behr B, Longaker MT, Quarto N. Differential activation of canonical Wnt signaling determines cranial sutures fate: a novel mechanism for sagittal suture craniosynostosis. Dev Biol. 2010;344(2):922-40.
Behr, B., Longaker, M. T., & Quarto, N. (2010). Differential activation of canonical Wnt signaling determines cranial sutures fate: a novel mechanism for sagittal suture craniosynostosis. Developmental Biology, 344(2), pp. 922-40. doi:10.1016/j.ydbio.2010.06.009.
Behr B, Longaker MT, Quarto N. Differential Activation of Canonical Wnt Signaling Determines Cranial Sutures Fate: a Novel Mechanism for Sagittal Suture Craniosynostosis. Dev Biol. 2010 Aug 15;344(2):922-40. PubMed PMID: 20547147.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Differential activation of canonical Wnt signaling determines cranial sutures fate: a novel mechanism for sagittal suture craniosynostosis. AU - Behr,Björn, AU - Longaker,Michael T, AU - Quarto,Natalina, Y1 - 2010/06/12/ PY - 2010/02/08/received PY - 2010/05/21/revised PY - 2010/06/04/accepted PY - 2010/6/16/entrez PY - 2010/6/16/pubmed PY - 2010/8/31/medline SP - 922 EP - 40 JF - Developmental biology JO - Dev. Biol. VL - 344 IS - 2 N2 - Premature closure of cranial sutures, which serve as growth centers for the skull vault, result in craniosynostosis. In the mouse posterior frontal (PF) suture closes by endochondral ossification, whereas sagittal (SAG) remain patent life time, although both are neural crest tissue derived. We therefore, investigated why cranial sutures of same tissue origin adopt a different fate. We demonstrated that closure of the PF suture is tightly regulated by canonical Wnt signaling, whereas patency of the SAG suture is achieved by constantly activated canonical Wnt signaling. Importantly, the fate of PF and SAG sutures can be reversed by manipulating Wnt signaling. Continuous activation of canonical Wnt signaling in the PF suture inhibits endochondral ossification and therefore, suture closure, In contrast, inhibition of canonical Wnt signaling in the SAG suture, upon treatment with Wnt antagonists results in endochondral ossification and suture closure. Thus, inhibition of canonical Wnt signaling in the SAG suture phenocopies craniosynostosis. Moreover, mice haploinsufficient for Twist1, a target gene of canonical Wnt signaling which inhibits chondrogenesis, have sagittal craniosynostosis. We propose that regulation of canonical Wnt signaling is of crucial importance during the physiological patterning of PF and SAG sutures. Importantly, dysregulation of this pathway may lead to craniosynostosis. SN - 1095-564X UR - https://www.unboundmedicine.com/medline/citation/20547147/Differential_activation_of_canonical_Wnt_signaling_determines_cranial_sutures_fate:_a_novel_mechanism_for_sagittal_suture_craniosynostosis_ L2 - https://linkinghub.elsevier.com/retrieve/pii/S0012-1606(10)00829-8 DB - PRIME DP - Unbound Medicine ER -