Additionally, severe gain-of-function mutations in KCNJ11 (Kir6.2) and ABCC8 (SUR1) are associated with developmental delay, epilepsy and neonatal diabetes (DEND).68-71 Neurodevelopmental abnormalities in AIMS may arise from neuronal and/or non-neuronal mechanisms, such as altered vascular or astrocyte dysfunction. The gene discussed is KCNJ11; the disease is epilepsy.