title: Fibulin-5 deficiency causes developmental defect of premaxillary bone in mice.
abstract: 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.
authors.affiliation: Department of Pediatrics, The University of Texas Medical School at Houston, Houston, TX 77030, USA. Electronic address: knoda@kuhp.kyoto-u.ac.jp.
authors.forename: Kazuo
authors.initials: K
authors.lastname: Noda
authors.affiliation: Department of Pharmacology, Kansai Medical University, Hirakata, Osaka 573-1010, Japan.
authors.forename: Tomoyuki
authors.initials: T
authors.lastname: Nakamura
authors.affiliation: Department of Pediatrics, The University of Texas Medical School at Houston, Houston, TX 77030, USA; Graduate Program in Genes and Development, The University of Texas Graduate School of Biomedical Sciences at Houston, Houston, TX 77030, USA.
authors.forename: Yoshihiro
authors.initials: Y
authors.lastname: Komatsu
mesh_terms: D000818:Animals; D002454:Cell Differentiation; D049109:Cell Proliferation; D003393:Cranial Sutures / Q000002:abnormalities / Q000378:metabolism / Q000473:pathology; D016326:Extracellular Matrix Proteins / Q000172:deficiency* / Q000235:genetics / Q000378:metabolism; D005260:Female; D008297:Male; D008437:Maxilla / Q000002:abnormalities* / Q000378:metabolism / Q000473:pathology; D008445:Maxillofacial Development; D059630:Mesenchymal Stem Cells / Q000378:metabolism / Q000473:pathology; D051379:Mice; D008810:Mice, Inbred C57BL; D018345:Mice, Knockout; D009432:Neural Crest / Q000378:metabolism / Q000473:pathology; D010006:Osteoblasts / Q000378:metabolism / Q000473:pathology; D012333:RNA, Messenger / Q000235:genetics / Q000378:metabolism; D011994:Recombinant Proteins / Q000235:genetics / Q000378:metabolism
chemical_list: D016326:Extracellular Matrix Proteins; C120769:Fbln5 protein, mouse; D012333:RNA, Messenger; D011994:Recombinant Proteins
keywords: Craniofacial development; Fibulin-5; Neural crest cells; Premaxillary bone; Suture