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To investigate the role of <jats:styled-content style=\"fixed-case\">LTBP<\/jats:styled-content>\u20103 in tooth formation we performed micro\u2010computed tomography (micro\u2010<jats:styled-content style=\"fixed-case\">CT<\/jats:styled-content>), histology, and scanning electron microscopy analyses of adult <jats:italic>Ltbp3\u2010\/\u2010<\/jats:italic> mice. The <jats:italic>Ltbp3\u2010\/\u2010<\/jats:italic> mutants presented with unique craniofacial malformations and reductions in enamel formation that began at the matrix formation stage. Organization of maturation\u2010stage ameloblasts was severely disrupted. The lateral side of the incisor was affected most. Reduced enamel mineralization, modification of the enamel prism pattern, and enamel nodules were observed throughout the incisors, as revealed by scanning electron microscopy. Molar roots had internal irregular bulbous\u2010like formations. The cementum thickness was reduced, and microscopic dentinal tubules showed minor nanostructural changes. Thus, <jats:styled-content style=\"fixed-case\">LTBP<\/jats:styled-content>\u20103 is required for ameloblast differentiation and for the formation of decussating enamel prisms, to prevent enamel nodule formation, and for proper root morphogenesis. Also, and consistent with the role of <jats:styled-content style=\"fixed-case\">TGF<\/jats:styled-content>\u2010<jats:italic>\u03b2<\/jats:italic> signaling during mineralization, almost all craniofacial bone components were affected in <jats:italic>Ltbp3\u2010\/\u2010<\/jats:italic> mice, especially those involving the upper jaw and snout. This mouse model demonstrates phenotypic overlap with Verloes Bourguignon syndrome, also caused by mutation of <jats:italic><jats:styled-content style=\"fixed-case\">LTBP<\/jats:styled-content>3<\/jats:italic>, which is hallmarked by craniofacial anomalies and amelogenesis imperfecta phenotypes.<\/jats:p>","DOI":"10.1111\/eos.12328","type":"journal-article","created":{"date-parts":[[2017,1,13]],"date-time":"2017-01-13T19:13:31Z","timestamp":1484334811000},"page":"8-17","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":15,"title":["Enamel and dental anomalies in latent\u2010transforming growth factor beta\u2010binding protein 3 mutant mice"],"prefix":"10.1111","volume":"125","author":[{"given":"Supawich","family":"Morkmued","sequence":"first","affiliation":[{"name":"Facult\u00e9 de Chirurgie Dentaire Universit\u00e9 de Strasbourg Strasbourg France"},{"name":"CNRS UMR_7104 INSERM U964 Institut de G\u00e9n\u00e9tique et de Biologie 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