Reduced Dentin Matrix Protein Expression in Camurati-Engelmann Disease Transgenic Mouse Model
| Autor: | Mary MacDougall, Philip Sohn, Xiangwei Wu, Christina M. Croney, Angela Gullard, Xu Cao, Olga A. Mamaeva |
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| Rok vydání: | 2015 |
| Předmět: |
0301 basic medicine
Physiology Clinical Biochemistry ALPL 030206 dentistry Cell Biology Anatomy Biology Bone morphogenetic protein Molecular biology DMP1 Extracellular matrix stomatognathic diseases 03 medical and health sciences 030104 developmental biology 0302 clinical medicine medicine.anatomical_structure stomatognathic system Dentin medicine MEPE Pulp (tooth) Dental alveolus |
| Zdroj: | Journal of Cellular Physiology. 231:1106-1113 |
| ISSN: | 0021-9541 |
| Popis: | Overexpression of transforming growth factor-β1 (TGF-β1) has been shown to lead to mineralization defects in both the enamel and dentin layers of teeth. A TGFB1 point mutation (H222D), derived from published cases of Camurati–Engelmann disease (CED), has been shown to constitutively activate TGF-β1, leading to excess bone matrix production. Although CED has been well documented in clinical case reports, there are no published studies on the effect of CED on the dentition. The objective of this study was to determine the dental manifestations of hyperactivated TGF-β1 signaling using an established mouse model of CED-derived TGF-β1 mutation. Murine dental tissues were studied via radiography, micro-CT, immunohistochemistry, and qRT-PCR. Results showed that initial decreased dental mineralized tissue density is resolved. Proliferation assays of incisor pulp and alveolar bone cell cultures revealed that cells from transgenic animals displayed a reduced rate of growth compared to alveolar bone cultures from wild-type mice. TGF-β family gene expression analysis indicated significant fold changes in the expression of Alpl, Bmp2–5, Col-1, -2, -4, and -6, Fgf, Mmp, Runx2, Tgfb3, Tfgbr3, and Vdr genes. Assessment of SIBLINGs revealed downregulation of Ibsp, Dmp1, Dspp, Mepe, and Spp1, as well as reduced staining for BMP-2 and VDR in mesenchymal-derived pulp tissue in CED animals. Treatment of dental pulp cells with recombinant human TGF-β1 resulted in increased SIBLING gene expression. Conclusions: Our results provide in vivo evidence suggesting that TFG-β1 mediates expression of important dentin extracellular matrix components secreted by dental pulp, and when unbalanced, may contribute to abnormal dentin disorders. J. Cell. Physiol. 231: 1106–1113, 2016. © 2015 Wiley Periodicals, Inc. |
| Databáze: | OpenAIRE |
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