Dentinogenesis imperfecta

Dentinogenesis imperfecta is an inherited disorder of tooth development that produces abnormally formed dentin, the layer beneath tooth enamel. Affected teeth typically appear discolored, most often blue-gray or yellow-brown, with a translucent or opalescent sheen, and have short, narrow roots and unusually small pulp chambers. Because the dentin is poorly mineralized, the teeth wear down and break easily, with baby teeth generally affected more severely than the permanent teeth that follow. The disorder is caused by mutations in the dentine sialophosphoprotein gene, DSPP, located on chromosome 4q21, and is passed down in an autosomal dominant pattern, making it one of the more frequently occurring dominant traits affecting human tooth structure. It affects an estimated one in 6,000 to 8,000 people.