Mutations in the DSPP gene in humans cause a non-syndromic inheritable dominant dental disorder, known as dentinogenesis imperfecta (DGI) (Shields et al., 1973; MacDougall et al., 2006; Kim and Simmer, 2007; Barron et al., 2008; McKnight et al., 2008; Nieminen et al., 2011; Li et al., 2012). Here, DSPP is linked to dentinogenesis imperfecta.