In humans, pathogenic mutations in SMOC2 have been implicated in severe dental anomalies and skeletal dysplasia, characterized by microdontia, dentin dysplasia, reduced alveolar/jaw bone density, and flatten lumbar vertebrae (Bloch-Zupan et al., 2011; Alfawaz et al., 2013). This evidence concerns the gene SMOC2 and dentin dysplasia.