SUR2 and Kir6.1 mutations cause essentially the same CS features, and we previously generated 2 mouse models of CS, in which human CS mutations (A478V in SUR2 and V65M in Kir6.1) were knocked in to the mouse genome using CRISPR/Cas9 (3, 17). This evidence concerns the gene ABCC9 and Cowden syndrome 1.