In unperturbed cells, the tumor suppressor NF2Merlin, an ezrin-moesin-radixin (ERM) family protein that is mutated in neurofibromatosis type 2, binds to and inhibits the nuclear CUL4-DCAF1VprBP complex (Trofatter et al. 1993; Rouleau et al. 1993; Cooper et al. 2011). Here, ETV5 is linked to NF2-related schwannomatosis.