On one side, autophagy defects are clearly involved, since different genes found mutated in ALS code for proteins of the autophagic system, like SQSTM1/p62, OPTN, VCP, UBQLN2, TBK1 and C9orf72 (Table 1; Nguyen et al., 2019). Here, SQSTM1 is linked to amyotrophic lateral sclerosis.