Here, we present the cryoEM structure of the KATP channel harboring the neonatal diabetes Q52R mutation (hereinafter referred to as the SUR1/Kir6.2Q52R channel) in an open conformation bound to amphipathic molecules consistent with natural C18:0/C20:4 long-chain PI(4,5)P2. This evidence concerns the gene ABCC8 and diabetes mellitus.