SQSTM1 encodes the SQSTM1/p62 protein and the overwhelming majority of mutations associated with PDB cluster within and around the C-terminal ubiquitin-associated (UBA) domain, whereas mutations associated with ALS-FTLD occur more widely throughout the protein sequence (Rea et al., 2014, Majcher et al., 2015). Here, SQSTM1 is linked to amyotrophic lateral sclerosis.