In addition to ectodermal dysplasia, CRAC channelopathies (STIM1 and ORAI1 deficiencies) are also characterized by enamel defects, such as hypomineralized amelogenesis imperfecta (7), which was not observed in our series of IP3R3 R2524C patients. Here, ITPR3 is linked to ectodermal dysplasia syndrome.