title: Tooth eruption and cementum formation in the Runx2/Cbfa1 heterozygous mouse.
abstract: 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.
authors.affiliation: Department of Oral Cell Biology, ACTA, van der Boechorststr 7, 1081 BT Amsterdam, The Netherlands.
authors.forename: S J
authors.initials: SJ
authors.lastname: Zou
authors.forename: R N
authors.initials: RN
authors.lastname: D'Souza
authors.forename: T
authors.initials: T
authors.lastname: Ahlberg
authors.forename: A L J J
authors.initials: AL
authors.lastname: Bronckers
mesh_terms: D000818:Animals; D025021:Cementogenesis / Q000235:genetics; D002973:Cleidocranial Dysplasia / Q000235:genetics* / Q000473:pathology / Q000503:physiopathology; D050659:Core Binding Factor Alpha 1 Subunit; D003739:Dental Cementum / Q000473:pathology*; D006579:Heterozygote; D051379:Mice; D008822:Mice, Transgenic; D008963:Molar / Q000254:growth & development* / Q000473:pathology; D009363:Neoplasm Proteins; D014078:Tooth Eruption / Q000235:genetics*; D014157:Transcription Factors / Q000235:genetics*
chemical_list: D050659:Core Binding Factor Alpha 1 Subunit; D009363:Neoplasm Proteins; D014157:Transcription Factors