Transgenic Mouse Model for Tooth Disorders such as Dentin Dysplasia and Dentinogenesis Imperfecta

This technology describes transgenic mice that selectively overexpress transforming growth factor beta-1 (TGF-beta1) in odontoblast and ameloblast cells of teeth. Ameloblasts mainly make enamel, whereas odontoblasts make dentin. These transgenic mice mimic dental symptoms similar to those seen in common tooth disorders such as dentin dysplasia and dentinogenesis imperfecta. Both of these human dentin defects are inherited in an autosomal dominant manner and appear to be caused by abnormal dentin production by odontoblasts and associated poor mineralization of the dentin matrix. In both diseases, teeth are discolored and fractured, causing difficulties in eating food. Experimentally, these mice display discolored and fractured teeth with defective dentin. This transgenic mice model will be valuable to advance our understanding of the molecular pathogenesis underlying dentin dysplasia and dentinogenesis imperfecta and also for developing therapeutic strategies.


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Updated: Jun 16, 2010