Tfap2α (previously known as AP-2α) and Pitx2 mutants show severe ventral body wall closure defects such as thoracoabdominoschisis and omphalocele (Zhang et al., 1996; Brewer and Williams, 2004a; Kitamura et al., 1999; Gage et al., 1999; Eng et al., 2012). Here, PITX2 is linked to omphalocele.