Further validation of the function of the KATP channel is provided by the excellent response of many patients harbouring KCNJ11/ABCC8 mutations to SU therapy; indeed, it is now standard practice for all patients with diabetes diagnosed before 6 months of age to undergo diagnostic testing for mutations in these two genes, with attempts to transfer those with confirmed mutations from insulin therapy to oral SUs [9, 46]. Here, INS is linked to diabetes mellitus.