This finding might have potential implications for the treatment of CS patients: glibenclamide reverses CS-associated cardiovascular pathologies in SUR2wt/A478V mutant mice but has reduced efficacy in Kir6.1wt/V65M mice, leading to the suggestion that clinical efficacy of glibenclamide may be limited in Kir6.1-variant CS patients [10,39,40]. Here, KCNJ8 is linked to Cowden syndrome 1.