Since the accumulation of autophagosome-like structures and SQSTM1-positive aggregates are among the cardinal pathological features in patients with ALS as well as in murine ALS models [18, 23], it is reasonable to assume that overexpression of SQSTM1 enhances mutant SOD1-associated neurotoxicity by further compromising proteostasis, thereby leading to an earlier onset of disease. Here, SOD1 is linked to amyotrophic lateral sclerosis.