Akin to other loss-of-function and gain-of-function genetic mouse models of ALS that affect components of the Ran GTPase cycle (Koppers et al., 2015; Peters et al., 2015; O'Rourke et al., 2015, 2016), loss of Ranbp2 in motoneurons did not promote their degeneration. Here, RANBP2 is linked to amyotrophic lateral sclerosis.