Quantification of three-dimensional morphology of craniofacial mineralized tissue defects in Tgfbr2/Osx-Cre mice.

Craniofacial morphology is affected by the growth, development, and three-dimensional (3D) relationship of mineralized structures including the skull, jaws, and teeth. Despite fulfilling different purposes within this region, cranial bones and tooth dentin are derived from mesenchymal cells that are affected by perturbations within the TGF-β signaling pathway. encodes a transmembrane receptor that is part of the canonical, SMAD-dependent TGF-β signaling pathway and mutations within this gene are associated with Loeys-Dietz syndrome, a condition which often presents with craniofacial signs including craniosynostosis and cleft palate. To investigate the role of in immature, but committed, mineralized tissue forming cells, we analyzed postnatal craniofacial morphology in mice with conditional deletion in -expressing cells. Novel application of a 3D shape-based comparative technique revealed that in -expressing cells results in impaired postnatal molar root and anterior cranial growth. These findings support those from studies using similar conditional knockout models, highlight the anomalous facial and dental regions/structures using tomographic imaging-based techniques, and provide insight into the role of during postnatal craniofacial development.