SQSTM1 and amyotrophic lateral sclerosis: The only mutant found to lack this ability was S349T, which as noted previously, unusually for PDB affects a region of the protein outside the UBA domain, specifically within the KIR and, as we report here for the ALS-associated P348L/G351A mutants, impacts on the interaction of SQSTM1/p62 with Keap1 (and on Nrf2 signalling) (Wright et al., 2013).