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]. The gene discussed is KCNJ8; the disease is hypertrichotic osteochondrodysplasia Cantu type.