In addition, encephaloceles are often observed to be associated with other human birth defects, including other NTDs, cleft palate, craniosynostosis (Naidich et al., 1992; Caplan et al., 2002; Ganapathy et al., 2014), which are similar phenotypes to those observed in mouse models with Gpr161 cKO or Rac1 cKO (Rolo et al., 2019). Here, RAC1 is linked to craniosynostosis.