Abstract

MARTIN-GONZALEZ, J. et al. Inherited dental abnormalities and dysplasias. Av Odontoestomatol [online]. 2012, vol.28, n.6, pp.287-301. ISSN 2340-3152.

Alterations of the embryologic development of the dentition cause dental anomalies and dysplasias. The causing factors involved in the disturbances of tooth development are basically two: genetic and environmental factors. Depending on the phase of tooth development when the factors act, they will appear different dental anomalies and dysplasias. Genetic control of tooth development is carried out through two processes: a) control of amelogenesis and dentinogenesis,and b) the specification of the type, size and position of each tooth. Mutation of genes involved in amelogenesis (AMELX, ENAM, MMP20 and KLK4) and dentinogenesis (DSPP) produces non-syndromic alterations of tooth development. On the contrary, mutations in morphogenetic regulatory genes involved in determining the position and the early development of the teeth (homeobox genes), not only alter teeth morpho differentiation, but also have pleiotropic effects affecting other organs, causing hereditary syndromes in which one of its features is the presence of dental abnormalities. Dental anomalies and dysplasias of genetic origin are reviewed in this article.

Keywords : Dental anomalies; dental dysplasia; amelogenesis; dentinogenesis; rare disease; homeobox gene.

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