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Cementum and dentin in hypophosphatasia
T van den Bos1, G Handoko, A Niehof
1Department of Periodontology, Academic Center for Dentistry Amsterdam (ACTA), Universiteit van Amsterdam, and Vrije Universiteit, Louwesweg 1, 1066 EA Amsterdam, The Netherlands.
Journal of Dental Research
|October 26, 2005
Summary
Hypophosphatasia (HPP) affects cementum formation, leading to premature tooth loss. Dentin is not similarly affected, suggesting pyrophosphate
Area of Science:
- Dental Science
- Biochemistry
- Mineralization
Background:
- Hypophosphatasia (HPP) is a genetic disorder characterized by defective bone and tooth mineralization.
- Premature loss of deciduous teeth is a common clinical manifestation of HPP, linked to impaired cementum formation.
- The differential impact of HPP on cementum versus dentin requires further investigation.
Purpose of the Study:
- To compare the effects of HPP on cementum and dentin formation.
- To investigate the role of pyrophosphate and related enzymes in the differential mineralization defects observed in HPP.
Main Methods:
- Microscopic and chemical analysis of teeth from children with HPP and matched controls.
- Assessment of pyrophosphate concentration and nucleotide pyrophosphatase phosphodiesterase 1 (NPP1) expression and activity in periodontal ligament and pulp tissues.
Main Results:
- Both acellular and cellular cementum formation were significantly affected in HPP teeth.
- No differences in dentin mineral content were observed between HPP and control teeth.
- Lower NPP1 expression, reduced NPP1 activity, and lower pyrophosphate concentration were found in pulp compared to periodontal ligament in normal teeth.
Conclusions:
- Cementum mineralization is more susceptible to the inhibitory effects of pyrophosphate than dentin mineralization in Hypophosphatasia.
- The distinct roles of pyrophosphate metabolism in pulp and periodontal ligament may explain the differential effects on dentin and cementum in HPP.
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