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Enamel structure and composition in the tricho-dento-osseous syndrome
G S Spangler1, K I Hall, K Kula
1School of Dentistry, The University of North Carolina at Chapel Hill, USA.
Connective Tissue Research
|November 4, 2000
Summary
Tricho-dento-osseous syndrome (TDO) causes significant enamel defects, including reduced thickness and lower mineral content in teeth. This genetic disorder highlights shared developmental pathways between hair, teeth, and bone.
Area of Science:
- Genetics
- Developmental Biology
- Dental Science
Background:
- Tricho-dento-osseous syndrome (TDO) is an autosomal dominant disorder.
- TDO is characterized by curly hair, hypoplastic enamel, taurodontism, and dense bone.
Purpose of the Study:
- To characterize the specific enamel defects in individuals with TDO.
- To investigate the microstructural and mineralogical properties of TDO enamel.
Main Methods:
- Examination of 12 TDO teeth and 12 normal control teeth.
- Analysis of enamel thickness, structure (prismless vs. prismatic), and crystallite morphology using Transmission Electron Microscopy (TEM).
- Quantification of mineral content per volume in TDO and normal enamel samples.
Main Results:
- TDO teeth exhibited reduced enamel thickness, ranging from absent to 60% of normal thickness.
- Half of TDO teeth showed primarily prismless enamel; the remainder had some prismatic areas.
- TDO enamel had significantly lower mineral content (68.5%) compared to normal enamel (84.5%).
- TEM revealed similar crystallite morphology between TDO and normal enamel.
Conclusions:
- TDO is associated with significant enamel hypoplasia and reduced mineralization.
- The findings suggest alterations in enamel development pathways due to the TDO genetic mutation.
- This underscores shared molecular-level developmental controls across hair, teeth, and bone tissues.