However, VAPB expression is down-regulated in ALS8 patient-derived iPSC neurons due to reduced expression of the VAPBP56S mutant (Mitne-Neto et al., 2011), therefore it is more likely ALS-associated loss of function of VAPB may predispose motor neurons to ER stress (Kanekura et al., 2006; Gkogkas et al., 2008; Suzuki et al., 2009). Here, VAPB is linked to amyotrophic lateral sclerosis.