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Using CRISPR\/Cas9, we generated a\n                    <jats:italic>Dspp<\/jats:italic>\n                    <jats:sup>\u22121fs<\/jats:sup>\n                    mouse model by introducing a FLAG-tag followed by a single nucleotide deletion that translated 493 extraneous amino acids before termination. Developing incisors and\/or molars from this mouse and a\n                    <jats:italic>Dspp<\/jats:italic>\n                    <jats:sup>P19L<\/jats:sup>\n                    mouse were characterized by morphological assessment, bSEM, nanohardness testing, histological analysis, in situ hybridization and immunohistochemistry.\n                    <jats:italic>Dspp<\/jats:italic>\n                    <jats:sup>P19L<\/jats:sup>\n                    dentin contained dentinal tubules but grew slowly and was softer and less mineralized than the wild-type.\n                    <jats:italic>Dspp<\/jats:italic>\n                    <jats:sup>P19L<\/jats:sup>\n                    incisor enamel was softer than normal, while molar enamel showed reduced rod\/interrod definition.\n                    <jats:italic>Dspp<\/jats:italic>\n                    <jats:sup>\u22121fs<\/jats:sup>\n                    dentin formation was analogous to reparative dentin: it lacked dentinal tubules, contained cellular debris, and was significantly softer and thinner than\n                    <jats:italic>Dspp<\/jats:italic>\n                    <jats:sup>+\/+<\/jats:sup>\n                    and\n                    <jats:italic>Dspp<\/jats:italic>\n                    <jats:sup>P19L<\/jats:sup>\n                    dentin. The\n                    <jats:italic>Dspp<\/jats:italic>\n                    <jats:sup>\u22121fs<\/jats:sup>\n                    incisor enamel appeared normal and was comparable to the wild-type in hardness. We conclude that 5\u2032 and 3\u2032\n                    <jats:italic>Dspp<\/jats:italic>\n                    mutations cause dental malformations through different pathological mechanisms and can be regarded as distinct disorders.\n                  <\/jats:p>","DOI":"10.1038\/s41598-021-00219-4","type":"journal-article","created":{"date-parts":[[2021,10,19]],"date-time":"2021-10-19T06:04:50Z","timestamp":1634623490000},"update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":18,"title":["Mouse Dspp frameshift model of human dentinogenesis imperfecta"],"prefix":"10.1038","volume":"11","author":[{"given":"Tian","family":"Liang","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yuanyuan","family":"Hu","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hong","family":"Zhang","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Qian","family":"Xu","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Charles E.","family":"Smith","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Chuhua","family":"Zhang","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jung-Wook","family":"Kim","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Shih-Kai","family":"Wang","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Thomas L.","family":"Saunders","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yongbo","family":"Lu","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jan C.-C.","family":"Hu","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"James P.","family":"Simmer","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2021,10,19]]},"reference":[{"key":"219_CR1","doi-asserted-by":"publisher","first-page":"5931","DOI":"10.1016\/S0021-9258(19)70720-0","volume":"255","author":"A Linde","year":"1980","unstructured":"Linde, A., Bhown, M. & Butler, W. 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