LRP4 mutations, dental anomalies, and oral exostoses.
Piranit Nik KantaputraWeena PanichkulParisri SillapasornPloy AdisornkanjPanita KitsadayurachMassupa KaewgayaWorrachet IntachaiBjorn OlsenChumpol NgamphiwChidchanok LeethanakulPeeranat JatooratthawichotJames R Ketudat CairnsSissades TongsimaPublished in: International journal of paediatric dentistry (2023)
Antagonism of Bmp signaling by Sostdc1 requires the presence of Lrp4. Mice lacking Lrp4 have been demonstrated to have alteration of Wnt-Bmp-Shh signaling and an abnormal number of incisors. Therefore, the LRP4 mutations found in our patients may disrupt Wnt-Bmp-Shh signaling, thereby resulting in dental anomalies and oral exostoses. Root maldevelopment in the patients suggests an important role of LRP4 in root morphogenesis.