The role of autophagy in ALS is illustrated through a small proportion of familial ALS cases due to pathogenic variants in the autophagy receptors SQSTM1 [160], OPTN [161], and UBQLN2 (ubiquilin 2) [162], all of which facilitate cargo recruitment into phagophores through their interactions with LC3. Here, SQSTM1 is linked to amyotrophic lateral sclerosis.