LZTR1, which is an adaptor for the E3 ubiquitin ligase CUL3, is recurrently altered in schwannomatosis (Piotrowski et al., 2014) and was identified as a tumor suppressor in glioblastoma multiforme (Frattini et al., 2013), and to associate with both autosomal-dominant and autosomal-recessive forms of NS. The gene discussed is LZTR1; the disease is Netherton syndrome.