- - a. imperfecta a group of hereditary ectodermal disorders in which the enamel is defective in structure or deficient in quantity. Three major groups are recognized: hypoplastic types, with defective enamel matrix deposition but normal mineralization; hypomineralization types, with normal matrix but defective mineralization; and hypomaturation type, in which the enamel crystallites remain immature. The several types may be inherited as autosomal dominant [MIM*104500, 104510, 104530], recessive [MIM*204650, 204690, 204700] or X-linked [MIM*301100, 301200, 301201]. SYN: enamel dysplasia, enamelogenesis imperfecta.
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* * *n.the formation of enamel by ameloblast, a process that is completed before tooth eruption. Amelogenesis imperfecta is a hereditary condition in which enamel formation is disturbed. The teeth have an unusual surface but may not be more prone to decay.
* * *am·e·lo·gen·e·sis (am″ə-lo-jenґə-sis) [amelo- + genesis] the elaboration of dental enamel by ameloblasts.
Medical dictionary. 2011.