An increase in basal KATP channel activity reduces insulin secretion and leads to neonatal diabetes. Mouse models of increased KATP channel activity display hyperglycaemia and hypoinsulinaemia: they include those with Kir6.2-V59M, Kir6.2-ΔN30, and Kir6.2-ΔN30,K185Q mutations. Reduced basal KATP channel activity results in congenital hyperinsulinism. Mouse models with partial deletion (Kir6.2+/-, SUR+/-) or ablated channel expression / function (Kir6.2-/-, SUR-/-, Kir6.2-Y12X, Kir6.2-AAA) have been generated but do not fully recapitulate the human disease.