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Tooth eruption and cementum formation in the Runx2/Cbfa1 heterozygous mouse

Cleidocranial dysplasia (CCD) is an autosomal dominant human disorder that affects development of bones and teeth. The dental disorders in CCD patients include formation of supernumerary teeth, delayed tooth eruption, and lack of formation of cellular cementum in permanent teeth. This disorder invol...

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Published in:Archives of oral biology 2003-09, Vol.48 (9), p.673-677
Main Authors: Zou, S.J, D’Souza, R.N, Ahlberg, T, Bronckers, A.L.J.J
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description Cleidocranial dysplasia (CCD) is an autosomal dominant human disorder that affects development of bones and teeth. The dental disorders in CCD patients include formation of supernumerary teeth, delayed tooth eruption, and lack of formation of cellular cementum in permanent teeth. This disorder involves a mutation in the osteoblast-specific transcription factor Runx2/Cbfa1, leading to haploinsufficiency of the Runx2/Cbfa1 protein. Here, we examined if Runx2/Cbfa1 heterozygous mice (with one functional allele for Runx2/Cbfa1) exhibit similar changes in tooth eruption, and dental cementum formation as in CCD patients. Heads of Runx2/Cbfa1 heterogeneous and wildtype mice aged days 16–35 postnatally were serially sectioned and stained with hematoxylin–eosin or for tartrate resistant acid phosphatase (TRAP) to identify osteoclasts. The results showed that the eruption pattern of the first and second molars in maxilla and mandible in Runx2/Cbfa1 +/− mice was the same as in wildtype animals. No clear difference in distribution or in the (estimated) number of osteoclasts was found. Cellular cement at the apical portions of the molar roots was present in both groups. The data suggests that in the mouse one allele for Runx2/Cbfa1 is sufficient for an undisturbed tooth eruption and an apparently normal formation of the periodontium.
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The dental disorders in CCD patients include formation of supernumerary teeth, delayed tooth eruption, and lack of formation of cellular cementum in permanent teeth. This disorder involves a mutation in the osteoblast-specific transcription factor Runx2/Cbfa1, leading to haploinsufficiency of the Runx2/Cbfa1 protein. Here, we examined if Runx2/Cbfa1 heterozygous mice (with one functional allele for Runx2/Cbfa1) exhibit similar changes in tooth eruption, and dental cementum formation as in CCD patients. Heads of Runx2/Cbfa1 heterogeneous and wildtype mice aged days 16–35 postnatally were serially sectioned and stained with hematoxylin–eosin or for tartrate resistant acid phosphatase (TRAP) to identify osteoclasts. The results showed that the eruption pattern of the first and second molars in maxilla and mandible in Runx2/Cbfa1 +/− mice was the same as in wildtype animals. No clear difference in distribution or in the (estimated) number of osteoclasts was found. 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subjects Animals
Cementogenesis - genetics
Cleidocranial dysplasia
Cleidocranial Dysplasia - genetics
Cleidocranial Dysplasia - pathology
Cleidocranial Dysplasia - physiopathology
Core Binding Factor Alpha 1 Subunit
Dental Cementum - pathology
Dentistry
Heterozygote
Mice
Mice, Transgenic
Molar - growth & development
Molar - pathology
Neoplasm Proteins
Periodontium
Tartrate resistant acid phosphatase
Tooth Eruption - genetics
Transcription factor
Transcription Factors - genetics
title Tooth eruption and cementum formation in the Runx2/Cbfa1 heterozygous mouse
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