{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,1,9]],"date-time":"2026-01-09T02:12:26Z","timestamp":1767924746070,"version":"3.49.0"},"reference-count":48,"publisher":"Oxford University Press (OUP)","issue":"8","license":[{"start":{"date-parts":[[2012,4,16]],"date-time":"2012-04-16T00:00:00Z","timestamp":1334534400000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/academic.oup.com\/journals\/pages\/open_access\/funder_policies\/chorus\/standard_publication_model"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2012,8,1]]},"abstract":"<jats:title>Abstract<\/jats:title>\n               <jats:p>Dentinogenesis imperfecta (DGI) is a hereditary defect of dentin, a calcified tissue that is the most abundant component of teeth. Most commonly, DGI is manifested as a part of osteogenesis imperfecta (OI) or the phenotype is restricted to dental findings only. In the latter case, DGI is caused by mutations in the DSPP gene, which codes for dentin sialoprotein (DSP) and dentin phosphoprotein (DPP). Although these two proteins together constitute the majority of noncollagenous proteins of the dentin, little is known about their transcriptional regulation. Here we demonstrate that mice overexpressing the Trps1 transcription factor (Col1a1-Trps1 mice) in dentin-producing cells, odontoblasts, present with severe defects of dentin formation that resemble DGI. Combined micro\u2013computed tomography (\u00b5CT) and histological analyses revealed tooth fragility due to severe hypomineralization of dentin and a diminished dentin layer with irregular mineralization in Col1a1-Trps1 mice. Biochemical analyses of noncollagenous dentin matrix proteins demonstrated decreased levels of both DSP and DPP proteins in Col1a1-Trps1 mice. On the molecular level, we demonstrated that sustained high levels of Trps1 in odontoblasts lead to dramatic decrease of Dspp expression as a result of direct inhibition of the Dspp promoter by Trps1. During tooth development Trps1 is highly expressed in preodontoblasts, but in mature odontoblasts secreting matrix its expression significantly decreases, which suggests a Trps1 role in odontoblast development. In these studies we identified Trps1 as a potent inhibitor of Dspp expression and the subsequent mineralization of dentin. Thus, we provide novel insights into mechanisms of transcriptional dysregulation that leads to DGI. \u00a9 2012 American Society for Bone and Mineral Research.<\/jats:p>","DOI":"10.1002\/jbmr.1636","type":"journal-article","created":{"date-parts":[[2012,4,16]],"date-time":"2012-04-16T19:40:40Z","timestamp":1334605240000},"page":"1735-1745","source":"Crossref","is-referenced-by-count":20,"title":["Transcriptional repression of the <i>Dspp<\/i> gene leads to dentinogenesis imperfecta phenotype in <i>Col1a1-Trps1<\/i> transgenic mice"],"prefix":"10.1093","volume":"27","author":[{"given":"Dobrawa","family":"Napierala","sequence":"first","affiliation":[{"name":"Department of Molecular and Human Genetics, Baylor College of Medicine, Houston, TX, USA"},{"name":"Institute of Oral Health Research, Department of Oral and Maxillofacial Surgery, School of Dentistry, University of Alabama at Birmingham, Birmingham, AL, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Yao","family":"Sun","sequence":"additional","affiliation":[{"name":"Institute of Hard Tissue Development and Regeneration, Harbin Medical University, Second Affiliate Hospital of Harbin Medical University, 246 Xuefu Road, Nangang, Harbin, China, 150086"},{"name":"Department of Biomedical Sciences, Baylor College of Dentistry, Texas A&M University Health Science Center, Dallas, TX, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Izabela","family":"Maciejewska","sequence":"additional","affiliation":[{"name":"Department of Dental Prosthetics, Medical University of Gdansk, Gdansk, Poland"},{"name":"Department of Biomedical Sciences, Baylor College of Dentistry, Texas A&M University Health Science Center, Dallas, TX, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Terry K","family":"Bertin","sequence":"additional","affiliation":[{"name":"Department of Molecular and Human Genetics, Baylor College of Medicine, Houston, TX, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Brian","family":"Dawson","sequence":"additional","affiliation":[{"name":"Howard Hughes Medical Institute, Baylor College of Medicine, Houston, TX, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Rena","family":"D'Souza","sequence":"additional","affiliation":[{"name":"Department of Biomedical Sciences, Baylor College of Dentistry, Texas A&M University Health Science Center, Dallas, TX, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Chunlin","family":"Qin","sequence":"additional","affiliation":[{"name":"Department of Biomedical Sciences, Baylor College of Dentistry, Texas A&M University Health Science Center, Dallas, TX, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Brendan","family":"Lee","sequence":"additional","affiliation":[{"name":"Howard Hughes Medical Institute, Baylor College of Medicine, Houston, TX, USA"},{"name":"Department of Molecular and Human Genetics, Baylor College of Medicine, Houston, TX, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"286","published-online":{"date-parts":[[2012,4,16]]},"reference":[{"key":"2024020318273544700_bib1","first-page":"1","volume-title":"Seltzer and Bender's dental pulp","author":"D'Souza","year":"2012","edition":"2nd ed"},{"key":"2024020318273544700_bib2","doi-asserted-by":"crossref","first-page":"1367","DOI":"10.1016\/j.biocel.2004.01.006","article-title":"Odontoblasts: the cells forming and maintaining dentine","volume":"8","author":"Arana-Chavez","year":"2004","journal-title":"Int J Biochem Cell Biol."},{"key":"2024020318273544700_bib3","doi-asserted-by":"crossref","first-page":"331","DOI":"10.1177\/10454411010120040401","article-title":"Proteoglycans in dentinogenesis","volume":"4","author":"Embery","year":"2001","journal-title":"Crit Rev Oral Biol 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