...Elaheh Dalir Abdolahinia; Sadaf Ilbeygi Taher; Parnaz Abdali Dehdezi; Atefe Ataei; Majid Azizi; Narges Afra; Samira Afshar Fard; Simin Sharifi Enamel tissue, the hardest body tissue, which covers the outside of the tooth shields the living tissue, but it erodes and disintegrates in the acidic...

...Yong Li; William S. Konicki; J. Timothy Wright; Cynthia Suggs; Hui Xue; Melissa A. Kuehl; Ashok B. Kulkarni; Carolyn W. Gibson Dental enamel covers the crown of the vertebrate tooth and is considered to be the hardest tissue in the body. Enamel develops during secretion of an extracellular matrix...

...Thomas Liam Coxon; Alan Henry Brook; Martin John Barron; Richard Nigel Smith Mutations in human and in mouse orthologous genes Amelx and Enam result in a diverse range of enamel defects. In this study we aimed to investigate the phenotype-genotype correlation between the mutants and the wild-type...

... + /Ca 2+ ) exchangers, which include the NCX and NCKX [sodium/calcium-potassium (Na + /Ca 2+ -K + ) exchanger] proteins. During enamel maturation, when crystals expand in thickness, Ca 2+ requirements vastly increase but exactly how Ca 2+ traffics through ameloblasts remains uncertain. Previous studies...

... to in the content or advertisements. Enamel Amelogenesis imperfecta FAM83H Mutation Amelogenesis imperfecta (AI) is a group of disorders with obvious clinical and genetic heterogeneity, causing abnormalities in the amount, structure and composition of enamel. They can occur as an isolated...

...Eszter Somogyi-Ganss; Yohei Nakayama; Kengo Iwasaki; Yukiko Nakano; Daiana Stolf; Marc D. McKee; Bernhard Ganss Tooth enamel is formed in a typical biomineralization process under the guidance of specific organic components. Amelotin (AMTN) is a recently identified, secreted protein...

...J. Timothy Wright; Melody Torain; Kimberly Long; Kim Seow; Peter Crawford; Michael J. Aldred; P. Suzanne Hart; Tom C. Hart Amelogenesis imperfecta (AI) represents hereditary conditions affecting the quality and quantity of enamel. Six genes are known to cause AI (AMELX, ENAM, MMP20, KLK4, FAM83H...

...E. Le Norcy; S.-Y. Kwak; F.B. Wiedemann-Bidlack; E. Beniash; Y. Yamakoshi; J.P. Simmer; H.C. Margolis N-terminal and C-terminal (CT) domains of amelogenin have been shown to be essential for proper enamel formation. Recent studies have also suggested that although the C-terminus plays an apparent...

... in Pro and Gln (P/Q) and are employed to form the tooth surface. In tetrapods, the tooth surface is usually covered with enamel which develops in a matrix comprised of P/Q-rich SCPPs. By contrast, the tooth surface tissue in teleosts is called enameloid and it forms in a dentin-like collagenous matrix...

... microscopy. The 3 strains expressed at either low (TgEGFP-RhoA DN -8), intermediate (TgEGFP-RhoA DN -2), or high (TgEGFP-RhoA DN -13) levels, and the molar teeth from the 3 strains had enamel hypoplasia and surface defects. We conclude that RhoA DN expressed in ameloblasts interferes with normal enamel...

... or services advertised or of their effectiveness, quality or safety. The publisher and the editor(s) disclaim responsibility for any injury to persons or property resulting from any ideas, methods, instructions or products referred to in the content or advertisements. Enamel Amelogenin Amelogenesis...

... expression and activity is frequently associated with an anion exchanger (AE) such as AE2 coded by the Slc4a2 gene. Mice null for Cftr and mice null for Slc4a2 have enamel defects, and there are some case reports of enamel anomalies in patients with CF. In this study we demonstrate that both Cftr and AE2...

...Daming Fan; Mayumi Iijima; Keith M. Bromley; Xiudong Yang; Shibi Mathew; Janet Moradian-Oldak Enamel matrix proteins, including the most abundant amelogenin and lesser amounts of enamelin, ameloblastin, and proteinases, play vital roles in controlling crystal nucleation and growth during enamel...

...W. El-Sayed; R.C. Shore; D.A. Parry; C.F. Inglehearn; A.J. Mighell Background: Mutations in WDR72 have been identified in autosomal recessive hypomaturation amelogenesis imperfecta (AI). Objective: to describe a novel WDR72 mutation and report the ultrastructural enamel phenotype associated...

..., sections of deciduous teeth from members of 3 of these families were examined by scanning electron microscopy (SEM) and the enamel mineral was analysed by energy dispersive X-ray spectroscopy (EDX). The sections were also probed with antibodies raised to a conserved sequence of the enamelin protein...

... with hypocalcified AI with small focal areas of more normal looking enamel. DNA sequencing identified a novel c.1374C>A p.Y458X FAM83H nonsense mutation in affected, but not in either unaffected family members or unrelated controls. Exfoliated teeth were characterised by substantial post-eruptive enamel loss...

... of the AmelX, Enam and Mmp20 genes have been generated. These mouse models provide tools for understanding enamel formation and AI pathogenesis. This study describes the AI phenotypes and relates them to their mouse model counterparts. Human AI phenotypes were determined in a clinical population of AI families...

...Coralee E. Tye; Rachel L. Lorenz; John D. Bartlett The enamel matrix proteins (amelogenin, enamelin and ameloblastin) are degraded by matrix metalloproteinase-20 and kallikrein-4 during enamel development and mature enamel is virtually protein free. The precise mechanism of removal and degradation...

...Carolyn W. Gibson; Yong Li; Bill Daly; Cynthia Suggs; Zhi-an Yuan; Hanson Fong; Darrin Simmons; Melissa Aragon; Ashok B. Kulkarni; J. Timothy Wright Introduction: The amelogenin proteins secreted by ameloblasts during dental enamel development are required for normal enamel structure. Amelx null...

... was altered in newborn and 7-day-old KO mice, but seemed normal in 21-day-old KO mice. DMP1 and DSPP may be involved in compensatory mechanisms. The enamel had a twisted appearance and looked porous at day 21 in KO incisor, and the outer aprismatic layer was missing in the molar. Alveolar bone formation...