Of all ALS cases, 90% are sporadic (sALS), while the remaining 10% are familial (fALS) and can be traced to mutations in a variety of genes, including the proteasome shuttle factor UBQLN2 (Deng et al., 2011; Gorrie et al., 2014; Williams et al., 2012). This evidence concerns the gene UBQLN2 and amyotrophic lateral sclerosis.