The P56S substitution almost entirely prevents VAPB binding to its FFAT-containing cytosolic partners; this contemporarily induces mutant VAPB aggregation both in cellular (Nishimura et al. 2004; Kanekura et al. 2006; Teuling et al. 2007; Suzuki et al. 2009) and in murine ALS models (Tudor et al. 2010; Qiu et al. 2013; Kuijpers et al. 2013a; Aliaga et al. 2013). Here, VAPB is linked to amyotrophic lateral sclerosis.