The Valosin Containing Protein (VCP), and Vesicle Associated Protein B (VAP B), which have been found mutated in small number of ALS cases, play a role in the clearance of misfolded proteins through the ER (ER associated degradation; ERAD) as well as proteasomal sorting of ubiquitinated proteins (Kuijpers et al., 2013; Meyer and Weihl, 2014; Moustaqim-Barrette et al., 2014). The gene discussed is VCP; the disease is amyotrophic lateral sclerosis.