Transgenic Mice that Express Normal and Mutated Amelogenins

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 amelogene...

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Bibliographic Details
Published in:Journal of dental research 2007-04, Vol.86 (4), p.331-335
Main Authors: Gibson, C.W., Yuan, Z.A., Li, Y., Daly, B., Suggs, C., Aragon, M.A., Alawi, F., Kulkarni, A.B., Wright, J.T.
Format: Article
Language:English
Subjects:
Amelogenesis - genetics
Amelogenesis Imperfecta - genetics
Amelogenin - genetics
Amino Acid Substitution
Animals
Dental Enamel - pathology
Female
Male
Mice
Mice, Transgenic
Mutagenesis, Site-Directed
Mutation, Missense
Odontogenic Tumors - genetics
Point Mutation
Proline - genetics
Threonine - genetics
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Summary: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.
ISSN:0022-0345
1544-0591
DOI:10.1177/154405910708600406