The role of NLRP6 has been also studied in individuals with Hirschsprung’s associated enterocolitis (HAEC), which is the principal cause of death in patients with Hirschsprung’s disease (HSCR), a congenital disorder characterized by the absence of ganglion cells at the end of the bowel69,70(Fig. 2a). Here, NLRP6 is linked to Hirschsprung disease.