Several mutations in p62/SQSTM1 have been identified in cases of familial and sporadic amyotrophic lateral sclerosis (ALS) and frontotemporal lobar degeneration (FTLD), leading to loss of function of p62/SQSTM1 in selective autophagy41,42, or a decreased interaction with Keap143. Here, SQSTM1 is linked to amyotrophic lateral sclerosis.