title: Enamel ultrastructure in pigmented hypomaturation amelogenesis imperfecta.
abstract: Hypomaturation amelogenesis imperfecta (AI) is a hereditary condition of enamel that is presumed to result from defects during the maturation stage of enamel development. This study characterized the enamel ultrastructure and enamel crystallite morphology, as well as the distribution of organic material in enamel affected with pigmented hypomaturation AI. Enamel exhibiting autosomal recessive pigmented hypomaturation AI was sectioned or fractured and examined using light microscopy, scanning electron microscopy and transmission electron microscopy. Enamel samples were treated with 30% NaOCl or 8 M urea to remove organic components and determine the effect of deproteinization on crystallite morphology. These were compared with untreated normal enamel samples. The enamel crystallites in hypomaturation AI exhibited considerable variability in size and morphology. Examination of deproteinized tissue indicated that the AI crystallites had a thick coating, presumably of organic or partially mineralized material, which was not visible in normal enamel. The results of this investigation provide further evidence that hypomaturation AI is associated with the retention of organic material that is most probably enamel protein. Enamel protein retention is likely to be involved in the inhibition of normal crystallite growth resulting in the morphological crystallite abnormalities associated with this disorder.
authors.affiliation: Department of Pediatric Dentistry, University of North Carolina, Chapel Hill.
authors.forename: J T
authors.initials: JT
authors.lastname: Wright
authors.forename: V
authors.initials: V
authors.lastname: Lord
authors.forename: C
authors.initials: C
authors.lastname: Robinson
authors.forename: R
authors.initials: R
authors.lastname: Shore
mesh_terms: D000567:Amelogenesis Imperfecta / Q000235:genetics / Q000473:pathology*; D002648:Child; D003461:Crystallography; D003743:Dental Enamel / Q000002:abnormalities / Q000648:ultrastructure*; D005260:Female; D005808:Genes, Recessive; D006801:Humans; D008854:Microscopy, Electron; D008855:Microscopy, Electron, Scanning; D010375:Pedigree; D010859:Pigmentation Disorders / Q000235:genetics / Q000473:pathology*