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Genetic Alterations: Heritable Dentin Defects

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The Dental Pulp

Abstract

Dentin defects that accompany rare genetic diseases (diseases that by definition affect less than 1/2,000 individuals) can be described phenotypically by various types of anomalies that not only impair the formation and structure of the dentin and per se the color of the teeth but also cause anomalies of the crown, root, and/or pulp space shape and aberrant dentin formation such as pulpolithes or intrapulpal calcifications. They may also induce or be associated with subsequent enamel, dentin/enamel junction, or cementum/periodontium anomalies as dentinogenesis proceeds and occurs in coordination and interaction with amelogenesis and periodontium formation through epithelio-mesenchymal interactions. In this chapter, we will describe the genetic alterations of genes encoding proteins involved in dentin and bone formation reflecting the similarities between the two tissue development and homeostasis and subsequent clinical phenotypes. Animal models presenting with dentinal defects will also be mentioned.

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Authors and Affiliations

  1. Department of Sciences Biologiques, Faculty of Dentistry, University of Strasbourg, 8 rue St. Elisabeth, Strasbourg, 67000, France

    Agnès Bloch-Zupan HDR, PhD, DDS

  2. Reference Center for Orodental Manifestations of Rare Diseases, Pôle de Médecine et Chirurgie Bucco-Dentaires, Hôpitaux Universitaires de Strasbourg, Strasbourg, France

    Agnès Bloch-Zupan HDR, PhD, DDS

  3. Institute of Genetics and Molecular and Cellular Biology, INSERM U964, CNRS-UdS UMR7104, Illkirch, France

    Agnès Bloch-Zupan HDR, PhD, DDS

  4. Eastman Dental Institute, UCL, London, UK

    Agnès Bloch-Zupan HDR, PhD, DDS

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  1. Agnès Bloch-Zupan HDR, PhD, DDS
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Correspondence to Agnès Bloch-Zupan HDR, PhD, DDS .

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  1. Department of Oral Biology, Institut National de la Santé et de la, Recherche Médicale, Université Paris Descartes, Paris, Paris, France

    Michel Goldberg

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Bloch-Zupan, A. (2014). Genetic Alterations: Heritable Dentin Defects. In: Goldberg, M. (eds) The Dental Pulp. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-642-55160-4_11

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