The effect of Angelman syndrome on enamel and dentin mineralization

Aims: To analyze the mineralization and ion content in deciduous, permanent teeth of Angelman syndrome in comparison to match-paired teeth from normal children. Methods: Three deciduous teeth and a third molar and a mesiodens extracted during routine dental treatment and their match-paired normal teeth were examined using energy dispersive X-ray spectrometer program under a scanning electron microscope. Results: The morphology of the enamel and dentin of Angelman syndrome (AS) teeth was similar to normal but the thickness of the enamel of deciduous canine and permanent teeth was reduced. The most marked differences were found in the enamel-in AS teeth, the enamel contained nitrogen in concentrations similar to dentin, implicating that the protein content of the enamel is different from normal teeth where nitrogen is absent. Conclusions: AS affects morphology and mineralization of enamel. It caused hypoplastic enamel and abnormal protein content in comparison to match-paired normal teeth. These findings show that AS also affects odontogenesis in addition to the known oral motor challenges.