Mice and humans affected by Cantu syndrome (CS), which is associated with the mutations of the KCNJ8 and ABCC9 genes encoding the Kir6.1 and SUR2 subunits, showed dysfunction of contractility throughout the intestine and death in the mice after the weaning on solid food [21]. This evidence concerns the gene ABCC9 and hypertrichotic osteochondrodysplasia Cantu type.