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Transgenic Mice that Express Normal and Mutated Amelogenins

¹ Dept. of Anatomy and Cell Biology and
³ Dept. of Pathology, School of Dental Medicine, University of Pennsylvania, 240 S. 40th Street, Philadelphia, PA 19104-6030, USA
² Dept. of Pediatric Dentistry, School of Dentistry, University of North Carolina, Chapel Hill, NC 27599, USA; and
⁴ Functional Genomics Section, National Institute of Dental and Craniofacial Research, NIH, Bethesda, MD 20892, USA

^* corresponding author, gibson{at}biochem.dental.upenn.edu

Amelogenin proteins are secreted by ameloblasts within the enamel organ during tooth development. To better understand the function of the 180-amino-acid amelogenin (M180), and to test the hypothesis that a single proline-to-threonine (P70T) change would lead to an enamel defect similar to amelogenesis imperfecta (AI) in humans, we generated transgenic mice with expression of M180, or M180 with the proline-to-threonine (P70T) mutation, under control of the Amelx gene regulatory regions. M180 teeth had a relatively normal phenotype; however, P70T mineral was abnormally porous, with aprismatic regions similar to those in enamel of male amelogenesis imperfecta patients with an identical mutation. When Amelx null females were mated with P70T transgenic males, offspring developed structures similar to calcifying epithelial odontogenic tumors in humans. The phenotype argues for dominant-negative activity for the P70T amelogenin, and for the robust nature of the process of amelogenesis.

KEY WORDS: dental enamel • amelogenin • transgenic mice • odontogenic tumor