Elevated WNT signaling and compromised Hedgehog signaling due to Evc2 loss of function contribute to the abnormal molar patterning

Ellis-van Creveld (EVC) syndrome is an autosomal recessive chondrodysplasia. The affected individuals bear a series of skeleton defects, congenital heart septum anomalies, midfacial and dental defects. Previous studies using Evc or Evc2 mutant mice have characterized the pathological mechanism leading to various types Some patients with EVC supernumerary tooth; however, it not known yet if there are tooth formed in mice, yes, what associated. In present study, we used analyze pattern molars at stages. Our demonstrate that loss function within mesenchymal cells leads abnormal molar patterning, most anterior mandible represents tooth. Finally, provide evidence supporting idea both compromised Hedgehog signaling elevated WNT due contributes formation.