Several of the mouse mutants with craniorachischisis have mutations that disrupt core components of the PCP pathway, including Celsr1, Vangl1, Vangl2, Fzd3, Fzd6, Dvl1, Dvl2 and Dvl3 (Kibar et al., 2001; Murdoch et al., 2001a; Hamblet et al., 2002; Curtin et al., 2003; Wang et al., 2006a; Wang et al., 2006b; Merte et al., 2010; Andre et al., 2012). Here, CELSR1 is linked to craniorachischisis.