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@ARTICLE{Nguyen:285021,
      author       = {Nguyen, T. N. and Ribes, S. and Andrique, C. and Requin, M.
                      and Bouchet, J. and Obtel, N. and Slimani, L. and Brouilly,
                      N. and Torrens, C. and Schmitt, A. and Guilbert, T. and
                      Morawietz, M. and Kiesow, A. and Brunelle, A. and Percot, A.
                      and Hadj-Rabia, S. and Gaucher, C. and Le Bivic, A. and
                      Houillier, P. and Bardet, C. and Muller, D. and Ramirez
                      Rozzi, F. and Coradin, T. and Breiderhoff, T. and Chaussain,
                      C.},
      title        = {{T}he {A}bsence of {C}laudin-10 in the {E}namel {O}rgan
                      {A}lters {I}ts {I}ntegrity.},
      journal      = {Journal of dental research},
      volume       = {105},
      number       = {3},
      issn         = {0022-0345},
      address      = {Thousand Oaks, Calif.},
      publisher    = {Sage},
      reportid     = {DZNE-2026-00146},
      pages        = {385 - 395},
      year         = {2026},
      abstract     = {Rare disorders related to tight junction (TJ) proteins have
                      been associated with amelogenesis imperfecta. Pathogenic
                      variants of CLDN10, encoding claudin-10b, a cation transport
                      pore, cause the autosomal recessive HELIX syndrome
                      (Hypohidrosis, Electrolyte imbalance, hypoLacrymia,
                      Ichthyosis, Xerostomia). Patients exhibit salivary
                      dysfunction and rapid enamel wear after tooth eruption.
                      Since Cldn10 is expressed in the dental epithelium, this
                      study explores the role of claudin-10b in amelogenesis. We
                      analyzed amelogenesis in constitutive and conditional Cldn10
                      knockout (KO) murine models, comparing the findings to human
                      HELIX enamel. First, analysis of constitutive Cldn10
                      knockout (KO) mice, which die within a few hours after
                      birth, showed that claudin-10 is present at the plasma
                      membrane of the stratum intermedium but not at the TJs
                      during the secretory stage. Its absence altered gene
                      expression related to ion transport and pH control, although
                      without major disturbance in cell polarization or enamel
                      matrix synthesis. Examination of later stages of
                      amelogenesis in epithelium-targeted conditional Cldn10 KO
                      mice showed that claudin-10 is present in the papillary
                      layer at the maturation stage. In its absence, the pH of the
                      enamel matrix was more basic during early maturation,
                      suggesting that claudin-10 determines enamel matrix pH.
                      However, at later stage of the maturation process, the pH
                      was corrected and the resulting enamel did not show major
                      structural or elemental alterations. These later findings
                      were confirmed by exploring the enamel of Cldn10 KO
                      transplanted tooth germs, which have developed in a
                      controlled mineral environment. Nevertheless, higher
                      contents of aluminum were detected in the enamel of
                      transplanted germs and in human HELIX enamel, suggesting
                      that claudin-10 deficiency may lead to a loss of enamel
                      organ integrity. These data suggest that while salivary
                      dysfunction is the main cause of enamel wear in HELIX,
                      claudin-10 plays a direct role in amelogenesis by
                      determining pH and enamel organ integrity.},
      keywords     = {Animals / Claudins: genetics / Claudins: physiology /
                      Claudins: deficiency / Mice, Knockout / Mice / Enamel Organ:
                      metabolism / Amelogenesis: genetics / Amelogenesis:
                      physiology / Hydrogen-Ion Concentration / Tight Junctions /
                      Dental Enamel / Humans / Aluminum (Other) / Ameloblasts
                      (Other) / Amelogenesis (Other) / Genetic disorders (Other) /
                      Ion transport (Other) / Tight junctions (Other) / Claudins
                      (NLM Chemicals) / claudin 10 (NLM Chemicals)},
      cin          = {AG Wurst},
      ddc          = {610},
      cid          = {I:(DE-2719)1140001},
      pnm          = {352 - Disease Mechanisms (POF4-352)},
      pid          = {G:(DE-HGF)POF4-352},
      typ          = {PUB:(DE-HGF)16},
      pubmed       = {pmid:40717352},
      doi          = {10.1177/00220345251349109},
      url          = {https://pub.dzne.de/record/285021},
}