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Enamel Formation and Amelogenesis Imperfecta
Dental enamel is the epithelial-derived hard tissue covering the crowns of teeth. It is the most highly mineralized and hardest tissue in the body. Dental enamel is acellular and has no physiologicalExpand
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Functions of KLK4 and MMP-20 in dental enamel formation
Abstract Two proteases are secreted into the enamel matrix of developing teeth. The early protease is enamelysin (MMP-20). The late protease is kallikrein 4 (KLK4). Mutations in MMP20 and KLK4 bothExpand
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Dentin Sialophosphoprotein Is Processed by MMP-2 and MMP-20 in Vitro and in Vivo*
Dentin sialophosphoprotein (DSPP) is a major secretory product of odontoblasts and is critical for proper tooth dentin formation. During dentinogenesis, DSPP is proteolytically cleaved into smallerExpand
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Enamelin and autosomal-dominant amelogenesis imperfecta.
  • J. Hu, Y. Yamakoshi
  • Biology, Medicine
  • Critical reviews in oral biology and medicine…
  • 1 November 2003
Dental enamel forms as a progressively thickening extracellular layer by the action of proteins secreted by ameloblasts. The most abundant enamel protein is amelogenin, which is expressed primarilyExpand
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Enamel Defects and Ameloblast-specific Expression in Enam Knock-out/lacZ Knock-in Mice*
  • J. Hu, Y. Hu, +8 authors J. Simmer
  • Chemistry, Medicine
  • Journal of Biological Chemistry
  • 18 April 2008
Enamelin is critical for proper dental enamel formation, and defects in the human enamelin gene cause autosomal dominant amelogenesis imperfecta. We used gene targeting to generate a knock-in mouseExpand
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ENAM Mutations in Autosomal-dominant Amelogenesis Imperfecta
To date, 4 unique enamelin gene (ENAM) defects have been identified in kindreds with amelogenesis imperfecta. To improve our understanding of the roles of enamelin in normal enamel formation, and toExpand
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MMP-20 mutation in autosomal recessive pigmented hypomaturation amelogenesis imperfecta
During mammalian tooth formation, two proteinases are secreted by ameloblasts: enamelysin (MMP-20) and kallikrein-4 (KLK4). Enamelysin is the early protease. It is expressed by ameloblasts throughoutExpand
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Hypomaturation Enamel Defects in Klk4 Knockout/LacZ Knockin Mice*
Kallikrein 4 (Klk4) is believed to play an essential role in enamel biomineralization, because defects in KLK4 cause hypomaturation amelogenesis imperfecta. We used gene targeting to generate aExpand
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Amelogenin p.M1T and p.W4S Mutations Underlying Hypoplastic X-linked Amelogenesis Imperfecta
Mutations in the human amelogenin gene (AMELX, Xp22.3) cause a phenotypically diverse set of inherited enamel malformations. We hypothesize that the effects of specific mutations on amelogeninExpand
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STIM1 and SLC24A4 Are Critical for Enamel Maturation
Dental enamel formation depends upon the transcellular transport of Ca2+ by ameloblasts, but little is known about the molecular mechanism, or even if the same process is operative during theExpand
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