Alexandria, Va., USA - At the 96th General Session of the International Association for Dental Research (IADR), held in conjunction with the IADR Pan European Regional (PER) Congress, Tian Liang, Texas A&M University, Dallas, USA gave an oral presentation titled "Unfolded Protein Response (UPR) Is Associated With Dentinogenesis Imperfecta (DGI)." The IADR/PER General Session & Exhibition is in London, England at the ExCeL London Convention Center from July 25-28, 2018.
Non-syndromic Dentinogenesis Imperfecta (DGI) is a disorder of tooth development that causes the teeth to be discolored, most often a blue-gray or yellow-brown color and translucent. Teeth are also weaker than normal, making them prone to rapid wear, breakage and loss. DGI is caused by mutations in the dentin sialophosphoprotein (DSPP) gene.
Previously, Liang and co-authors from the Texas A&M University generated a mouse model expressing a mouse equivalent of human mutant DSPP called "Dspp mutant." In this study, they investigated the roles of unfolded protein response (UPR) in mediating the pathogenic effects of mutant DSPP in the Dspp mutant mice.
In situ hybridization, quantitative PCR (qPCR) and immunohistochemistry were used to analyze Dspp expression and the three main branches of UPR, including inositol-requiring enzyme 1a (IRE1a), pancreatic ER eukaryotic translation initiation factor (eIF)-2a kinase. In situ hybridization and qPCR showed that the level of DSPP mRNA was dramatically reduced. Immunohistochemistry revealed that the DSP protein level was increased within the odontoblasts but decreased in the dentin matrix in the Dspp mutant mice. Further analyses demonstrated that all three UPR branches were activated in the Dspp mutant mice. These results suggest that chronic UPR might be associated with DGI caused by mutant DSPP.
This research was presented as part of the Mineralized Tissue 1 oral session that took place on Wednesday, July 25 from 3:15 p.m. - 4:45 p.m. at the ExCeL London Convention Center in London, England.