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http://purl.uniprot.org/citations/34630144http://www.w3.org/1999/02/22-rdf-syntax-ns#typehttp://purl.uniprot.org/core/Journal_Citation
http://purl.uniprot.org/citations/34630144http://www.w3.org/2000/01/rdf-schema#comment"Dentin sialophosphoprotein (DSPP) is an extracellular matrix protein that is highly expressed in odontoblasts, but only transiently expressed in presecretory ameloblasts during tooth development. We previously generated a knockin mouse model expressing a mouse equivalent (DSPP, p.P19L) of human mutant DSPP (p.P17L; referred to as "DsppP19L/+ "), and reported that DsppP19L/+ and DsppP19L/P19L mice manifested a dentin phenotype resembling human dentinogenesis imperfecta (DGI). In this study, we analyzed pathogenic effects of mutant P19L-DSPP on enamel development in DsppP19L/+ and DsppP19L/P19L mice. Micro-Computed Tomography (μCT) analyses of 7-week-old mouse mandibular incisors showed that DsppP19L/P19L mice had significantly decreased enamel volume and/or enamel density at different stages of amelogenesis examined. Acid-etched scanning electron microscopy (SEM) analyses of mouse incisors demonstrated that, at the mid-late maturation stage of amelogenesis, the enamel of wild-type mice already had apparent decussating pattern of enamel rods, whereas only minute particulates were found in DsppP19L/+ mice, and no discernible structures in DsppP19L/P19L mouse enamel. However, by the time that incisor enamel was about to erupt into oral cavity, distinct decussating enamel rods were evident in DsppP19L/+ mice, but only poorly-defined enamel rods were revealed in DsppP19L/P19L mice. Moreover, μCT analyses of the mandibular first molars showed that DsppP19L/+ and DsppP19L/P19L mice had a significant reduction in enamel volume and enamel density at the ages of 2, 3, and 24weeks after birth. Backscattered and acid-etched SEM analyses revealed that while 3-week-old DsppP19L/+ mice had similar pattern of enamel rods in the mandibular first molars as age-matched wild-type mice, no distinct enamel rods were observed in DsppP19L/P19L mice. Yet neither DsppP19L/+ nor DsppP19L/P19L mice showed well-defined enamel rods in the mandibular first molars by the age of 24weeks, as judged by backscattered and acid-etched SEM. In situ hybridization showed that DSPP mRNA level was markedly reduced in the presecretory ameloblasts, but immunohistochemistry revealed that DSP/DSPP immunostaining signals were much stronger within the presecretory ameloblasts in Dspp mutant mice than in wild-type mice. These results suggest that mutant P19L-DSPP protein caused developmental enamel defects in mice, which may be associated with intracellular retention of mutant DSPP in the presecretory ameloblasts."xsd:string
http://purl.uniprot.org/citations/34630144http://purl.org/dc/terms/identifier"doi:10.3389/fphys.2021.724098"xsd:string
http://purl.uniprot.org/citations/34630144http://purl.uniprot.org/core/author"Lu Y."xsd:string
http://purl.uniprot.org/citations/34630144http://purl.uniprot.org/core/author"Qin C."xsd:string
http://purl.uniprot.org/citations/34630144http://purl.uniprot.org/core/author"Wang S."xsd:string
http://purl.uniprot.org/citations/34630144http://purl.uniprot.org/core/author"Xu Q."xsd:string
http://purl.uniprot.org/citations/34630144http://purl.uniprot.org/core/author"Zhang H."xsd:string
http://purl.uniprot.org/citations/34630144http://purl.uniprot.org/core/author"Liang T."xsd:string
http://purl.uniprot.org/citations/34630144http://purl.uniprot.org/core/author"Diekwisch T.G.H."xsd:string
http://purl.uniprot.org/citations/34630144http://purl.uniprot.org/core/date"2021"xsd:gYear
http://purl.uniprot.org/citations/34630144http://purl.uniprot.org/core/name"Front Physiol"xsd:string
http://purl.uniprot.org/citations/34630144http://purl.uniprot.org/core/pages"724098"xsd:string
http://purl.uniprot.org/citations/34630144http://purl.uniprot.org/core/title"Enamel Defects Associated With Dentin Sialophosphoprotein Mutation in Mice."xsd:string
http://purl.uniprot.org/citations/34630144http://purl.uniprot.org/core/volume"12"xsd:string
http://purl.uniprot.org/citations/34630144http://www.w3.org/2004/02/skos/core#exactMatchhttp://purl.uniprot.org/pubmed/34630144
http://purl.uniprot.org/citations/34630144http://xmlns.com/foaf/0.1/primaryTopicOfhttps://pubmed.ncbi.nlm.nih.gov/34630144
http://purl.uniprot.org/uniprot/#_E9Q9Z9-mappedCitation-34630144http://www.w3.org/1999/02/22-rdf-syntax-ns#objecthttp://purl.uniprot.org/citations/34630144
http://purl.uniprot.org/uniprot/#_A1L2Z1-mappedCitation-34630144http://www.w3.org/1999/02/22-rdf-syntax-ns#objecthttp://purl.uniprot.org/citations/34630144
http://purl.uniprot.org/uniprot/#_P97399-mappedCitation-34630144http://www.w3.org/1999/02/22-rdf-syntax-ns#objecthttp://purl.uniprot.org/citations/34630144
http://purl.uniprot.org/uniprot/#_Q1JQ89-mappedCitation-34630144http://www.w3.org/1999/02/22-rdf-syntax-ns#objecthttp://purl.uniprot.org/citations/34630144
http://purl.uniprot.org/uniprot/P97399http://purl.uniprot.org/core/mappedCitationhttp://purl.uniprot.org/citations/34630144
http://purl.uniprot.org/uniprot/A1L2Z1http://purl.uniprot.org/core/mappedCitationhttp://purl.uniprot.org/citations/34630144
http://purl.uniprot.org/uniprot/E9Q9Z9http://purl.uniprot.org/core/mappedCitationhttp://purl.uniprot.org/citations/34630144
http://purl.uniprot.org/uniprot/Q1JQ89http://purl.uniprot.org/core/mappedCitationhttp://purl.uniprot.org/citations/34630144
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