Severe hypoglycemia is rarely observed in SU-treated KCNJ11 PNDM.5 6 This is remarkable given that the doses of SU used in affected individuals are around 5–10 times those used to treat type 2 diabetes (T2D) and indicates that SU-stimulated KATP channel activity is regulated, at least in part, in the presence of KCNJ11 mutations. The gene discussed is KCNJ11; the disease is type 2 diabetes mellitus.