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Amelogenesis Imperfecta and Dentinogenesis Imperfecta

  • Amelogenesis imperfecta and dentinogenesis imperfecta are linked to defects in structural genes that code for proteins essential to the development of tooth enamel (amelogenesis imperfecta) or dentin (dentinogenesis imperfecta).
  • The teeth are weak and extremely sensitive to temperature and pressure. The ordinary forces of chewing are painful and can lead to further wear and pain.
  • The enamel matrix genes include tuftelin, ameloblastin, and amelogenin; researchers have begun to link mutations in these genes with amelogenesis imperfecta. Similarly, genes labeledDSP and DPP have been characterized for dentin matrix and are associated with the inheritance of dentinogenesis imperfecta.


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