Recently, Gloyn et al. reported in an ethnically diverse patient cohort that six heterozygous activating mutations in the KCNJ11 gene encoding the Kir6.2 subunit of the pancreatic β-cell potassium ATP (KATP) channel caused PNDM in ten probands [18]. The gene discussed is KCNJ11; the disease is permanent neonatal diabetes mellitus.