Enamel structure and composition in the tricho-dento-osseous syndrome

G. S. Spangler, K. I. Hall, K. Kula, T. C. Hart, J. T. Wright

Research output: Contribution to journalArticle

Abstract

Tricho-dento-osseous syndrome (TDO) is an autosomal dominant disorder characterized by curly hair, hypoplastic enamel, taurodontism, and dense bone. The purpose of this investigation was to characterize the enamel defects in a TDO population in North Carolina. Twelve TDO teeth and 12 normal teeth were examined. The enamel thickness was decreased in all TDO teeth ranging from having no enamel to about 60% the thickness of normal teeth. Half of the TDO teeth had primarily prismless enamel while the remainder had at least occasional areas of prismatic enamel. TDO enamel crystallites appeared similar to normal crystallites with TEM. The mineral per volume of TDO enamel (n = 9) (68.5%) was significantly less, on average, compared with normal enamel (n = 8) (84.5). The genetic mutation responsible for the TDO phenotype results in alteration of a developmental pathway(s) common to hair, teeth and bone. This further illustrates that these embryologically diverse tissues share common developmental controls at the molecular level.

Original languageEnglish (US)
Pages (from-to)469-479
Number of pages11
JournalConnective Tissue Research
Volume39
Issue number1-3
StatePublished - 1998
Externally publishedYes

Keywords

  • Calcium
  • Hereditary
  • Hypoplasia
  • Mineral
  • Phosphorus
  • Teeth

ASJC Scopus subject areas

  • Biochemistry
  • Cell Biology
  • Immunology
  • Nephrology
  • Orthopedics and Sports Medicine

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  • Cite this

    Spangler, G. S., Hall, K. I., Kula, K., Hart, T. C., & Wright, J. T. (1998). Enamel structure and composition in the tricho-dento-osseous syndrome. Connective Tissue Research, 39(1-3), 469-479.