Enamelin is critical for ameloblast integrity and enamel ultrastructure formation.
Enamelin is critical for ameloblast integrity and enamel ultrastructure formation.
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DOI：
10.1371/journal.pone.0089303
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发表时间：
2014
期刊：
影响因子：
3.7
通讯作者：
Simmer JP
中科院分区：
文献类型：
--
作者：
Hu JC;Hu Y;Lu Y;Smith CE;Lertlam R;Wright JT;Suggs C;McKee MD;Beniash E;Kabir ME;Simmer JP
Mutations in the human enamelin gene cause autosomal dominant hypoplastic amelogenesis imperfecta in which the affected enamel is thin or absent. Study of enamelin knockout NLS-lacZ knockin mice revealed that mineralization along the distal membrane of ameloblast is deficient, resulting in no true enamel formation. To determine the function of enamelin during enamel formation, we characterized the developing teeth of the Enam−/− mice, generated amelogenin-driven enamelin transgenic mouse models, and then introduced enamelin transgenes into the Enam−/− mice to rescue enamel defects. Mice at specific stages of development were subjected to morphologic and structural analysis using β-galactosidase staining, immunohistochemistry, and transmission and scanning electron microscopy. Enamelin expression was ameloblast-specific. In the absence of enamelin, ameloblasts pathology became evident at the onset of the secretory stage. Although the aggregated ameloblasts generated matrix-containing amelogenin, they were not able to create a well-defined enamel space or produce normal enamel crystals. When enamelin is present at half of the normal quantity, enamel was thinner with enamel rods not as tightly arranged as in wild type suggesting that a specific quantity of enamelin is critical for normal enamel formation. Enamelin dosage effect was further demonstrated in transgenic mouse lines over expressing enamelin. Introducing enamelin transgene at various expression levels into the Enam−/− background did not fully recover enamel formation while a medium expresser in the Enam+/− background did. Too much or too little enamelin abolishes the production of enamel crystals and prism structure. Enamelin is essential for ameloblast integrity and enamel formation.
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影响因子：
--
作者：
Chan AH;Lertlam R;Simmer JP;Wang CN;Hu JC
通讯作者：
Hu JC
影响因子：
3.5
作者：
Masuya, H;Shimizu, K;Shiroishi, T
通讯作者：
Shiroishi, T
影响因子：
--
作者：
UCHIDA, T;TANABE, T;SHIMIZU, M
通讯作者：
SHIMIZU, M
影响因子：
4.5
作者：
Meredith RW;Gatesy J;Murphy WJ;Ryder OA;Springer MS
通讯作者：
Springer MS
影响因子：
14.9
作者：
Simmer, James P.;Richardson, Amelia S.;Hu, Yuan-Yuan;Smith, Charles E.;Hu, Jan Ching-Chun
通讯作者：
Hu, Jan Ching-Chun