Mutations in the autophagy receptors, ubiquilin-2, optineurin, and p62/sequestosome-1 have been shown to cause ALS (Fecto et al., 2011; Hirano et al., 2013; Teyssou et al., 2013; Chen et al., 2014; Majcher et al., 2015; Figure 7). Here, OPTN is linked to amyotrophic lateral sclerosis.