Nakamura [83] identified significantly downregulated gene expression levels of NLRP3, NLRP12, NLRC4, ASC, and pro-IL-1, and significantly reduced protein expression levels of NLRP3, NLRP12, NLRC4, and ASC, in the colonic epithelium of patients with HSCR. Here, NLRC4 is linked to Hirschsprung disease.