It is interesting to note that in the CDH model in which Gata4 is deleted in the muscle connective tissue fibroblasts using Prx1-Cre (Prx1CreTg/+;Gata4∇/flx;Rosa26LacZ/+), B-gal+ sacs are present covering herniated regions, and not as holes in this tissue (Merrell et al., 2015). Here, GATA4 is linked to congenital diaphragmatic hernia.