Activating heterozygous mutations in the genes encoding either of the subunits of the ATP-sensitive K+ channel (KATP channel; KCNJ11 or ABCC8) of the pancreatic beta cell are the second most common cause of TNDM (26% of TNDM cases) and the most common causes of PNDM (44% of PNDM cases) (2). Here, ABCC8 is linked to permanent neonatal diabetes mellitus.