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Dentin Alteration of Deciduous Teeth in Human Hypophosphatemic Rickets

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Abstract

Familial hypophosphatemic rickets is in most cases transmitted as an X-linked dominant trait and results from mutation of the PHEX gene, predominantly expressed in osteoblast and odontoblast. Patients have been reported to display important dentin defects, and therefore, we explored the dentin structure, composition, and distribution of extracellular matrix (ECM) molecules in hypophosphatemic human deciduous teeth. Compared to age-matched controls, the dentin from hypophosphatemic patients exhibited major differences: presence of large interglobular spaces resulting from the lack of fusion of calcospherites in the circumpulpal dentin; defective mineralization in the interglobular spaces contrasting with normal Ca-P levels in the calcospherites on X-ray microanalysis; abnormal presence of low-molecular weight protein complexes recognized on Western blots by antibodies against matrix extracellular phosphoglycoprotein (MEPE), dentin sialoprotein, osteopontin, and reduced osteocalcin (OC) level; and accumulation in the interglobular spaces of immunolabeling with antibodies against DSP, dentin matrix protein, bone sialoprotein, MEPE and OC, while chondroitin/dermatan sulfate glycosaminoglycans were exclusively located inside calcospherites. Alterations of the post-translational processing or partial degradation of some ECM appear as key factors in the formation of the defective hypophosphatemic dentin.

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Acknowledgment

We thank Dr. Caren Gundberg (Yale University, New Haven, CT) for fruitful discussion on OC, David Montero for help in the SEM and X-ray microanalysis, and the French Ministry of Education and Research for the support given to our laboratory (EA2496).

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Authors and Affiliations

  1. Laboratoire Réparation et Remodelage des Tissus Oro-Faciaux, EA 2496 Groupe Matrices extracellulaires et biominéralisations, Faculté de Chirurgie Dentaire, Université University Paris 5, 1 rue Maurice Arnoux, 92120, Montrouge, France

    T. Boukpessi, D. Septier, M. Goldberg & C. Chaussain-Miller

  2. Faculté de Chirurgie Dentaire de Monastir, Tunisie

    S. Bagga

  3. Department of Pediatric Endocrinology and INSERM U561, Hôpital St. Vincent de Paul, Paris, France

    M. Garabedian

Authors
  1. T. Boukpessi
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  2. D. Septier
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  3. S. Bagga
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  4. M. Garabedian
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  5. M. Goldberg
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  6. C. Chaussain-Miller
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Correspondence to C. Chaussain-Miller.

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Boukpessi, T., Septier, D., Bagga, S. et al. Dentin Alteration of Deciduous Teeth in Human Hypophosphatemic Rickets. Calcif Tissue Int 79, 294–300 (2006). https://doi.org/10.1007/s00223-006-0182-4

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  • DOI: https://doi.org/10.1007/s00223-006-0182-4

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