The involvement of such pathobiological pathways in ALS can also be substantiated by the recent findings of SQSTM1 (encoding the p62 protein, a multifunctional protein that binds ubiquitin and is one of the best-known autophagic substrates) (41) mutations both in ALS and FTD (24,25). Here, SQSTM1 is linked to amyotrophic lateral sclerosis.