Rat wct mutation induces a hypo-mineralization form of amelogenesis imperfecta and cyst formation in molar teeth

Our previous findings have demonstrated that the rat autosomal-recessive mutation, whitish chalk-like teeth (wct), induces enamel defects resembling those of human amelogenesis imperfecta (AI) in continuously growing incisor teeth. The present study clarifies the effect of the wct mutation on the morphogenesis and calcification of rat molar teeth. Formalin-fixed maxillae obtained from animals aged 4-30 days were examined by electron probe micro-analysis (EPMA) and by immunocytochemistry for amelogenin, ameloblastin, and enamelin. There were no distinct differences in the calcium and phosphorous contents and the amount of enamel between homozygous mutant and wild-type teeth during postnatal days 4–11. Although the mineral density in the enamel matrix considerably increased in the wild-type teeth until day 15, no changes occurred in mutant teeth during days 11–30. The immunoreactivity for enamel proteins in the secretory-stage ameloblasts in mutant teeth was similar to that in the wild-type teeth, and subsequently mutant maturation-stage ameloblasts became detached from the enamel surface, resulting in odontogenic cyst formation between the enamel organ and matrix until day 7 and the expansion of the cyst around the whole tooth crown on day 15. On day 30, the erupted mutant teeth presented morphological changes such as enamel destruction and tertiary dentin formation in addition to low mineral density in the enamel. Thus, the wct mutation prevents mineral transport without disturbing the synthesis of enamel proteins in molar teeth because of the absence of maturation-stage ameloblasts, in addition to the occurrence of odontogenic cysts. Electronic supplementary material The online version of this article (doi:) contains supplementary material, which is available to authorized users. This work was supported in part by KAKENHI (B) (no. 16390523 to H.O.) and KAKENHI (C) (no. 18592002 to T.U.) from MEXT, Japan.