Mutations in CLDN10, CLDN16, and CLDN19 are associated with amelogenesis imperfecta in humans (Bardet et al., 2016; Yamaguti et al., 2017; Kim et al., 2020) and in mice, deletion of Cldn3 significantly reduces the enamel volume compared to that of wild-type mice (Bardet et al., 2017). Here, CLDN16 is linked to amelogenesis imperfecta.