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Publication : Phenotypic changes in dentition of Runx2 homozygote-null mutant mice.

First Author  Aberg T Year  2004
Journal  J Histochem Cytochem Volume  52
Issue  1 Pages  131-9
PubMed ID  14688224 Mgi Jnum  J:87161
Mgi Id  MGI:2683810 Doi  10.1177/002215540405200113
Citation  Aberg T, et al. (2004) Phenotypic changes in dentition of Runx2 homozygote-null mutant mice. J Histochem Cytochem 52(1):131-9
abstractText  Genetic and molecular studies in humans and mice indicate that Runx2 (Cbfa1) is a critical transcriptional regulator of bone and tooth formation. Heterozygous mutations in Runx2 cause cleidocranial dysplasia (CCD), an inherited disorder in humans and mice characterized by skeletal defects, supernumerary teeth, and delayed eruption. Mice lacking the Runx2 gene die at birth and lack bone and tooth development. Our extended phenotypic studies of Runx2 mutants showed that developing teeth fail to advance beyond the bud stage and that mandibular molar organs were more severely affected than maxillary molar organs. Runx2 (-/-) tooth organs, when transplanted beneath the kidney capsules of nude mice, failed to progress in development. Tooth epithelial-mesenchymal recombinations using Runx2 (+/+) and (-/-) tissues indicate that the defect in mesenchyme cannot be rescued by normal dental epithelium. Finally, our molecular analyses showed differential effects of the absence of Runx2 on tooth extracellular matrix (ECM) gene expression. These data support the hypothesis that Runx2 is one of the key mesenchymal factors that influences tooth morphogenesis and the subsequent differentiation of ameloblasts and odontoblasts.
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