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Publication : Fibulin-5 deficiency causes developmental defect of premaxillary bone in mice.

First Author  Noda K Year  2015
Journal  Biochem Biophys Res Commun Volume  466
Issue  3 Pages  585-91
PubMed ID  26399686 Mgi Jnum  J:232938
Mgi Id  MGI:5780490 Doi  10.1016/j.bbrc.2015.09.089
Citation  Noda K, et al. (2015) Fibulin-5 deficiency causes developmental defect of premaxillary bone in mice. Biochem Biophys Res Commun 466(3):585-91
abstractText  Craniofacial sutures govern the shape of the craniofacial skeleton during postnatal development. The differentiation of suture mesenchymal cells to osteoblasts is precisely regulated in part by signaling through cell surface receptors that interact with extracellular proteins. Here we report that fibulin-5, a key extracellular matrix protein, is important for craniofacial skeletal development in mice. Fibulin-5 is deposited as a fibrous matrix in cranial neural crest-derived mesenchymal tissues, including craniofacial sutures. Fibulin-5-null mice show decreased premaxillary bone outgrowth during postnatal stages. While premaxillo-maxillary suture mesenchymal cells in fibulin-5-null mice were capable of differentiating into osteoblasts, suture cells in mutant mice were less proliferative. Our study provides the first evidence that fibulin-5 is indispensable for the regulation of facial suture mesenchymal cell proliferation required for craniofacial skeletal morphogenesis.
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