Arising from these efforts, mouse models of TSC1- or TSC2-deficiency now exist that closely model the CNS manifestations of TSC that occur in human patients, including neuronal network dysfunction and seizure activity, sub-ependymal nodules that lead to the development of SEGAs and elements of cortical tubers, accompanied by abnormal neuronal migration, enhanced astrogliosis, enlarged cells and cortical lamination defects (Goto et al., 2011; Magri et al., 2011; Carson et al., 2012; Magri and Galli, 2013; Normand et al., 2013; Prabhakar et al., 2013). The gene discussed is TSC2; the disease is tuberous sclerosis.