In fact, mutations in the ubiquitin-binding site on p62/SQSTM1 have been associated with ALS and FTLD caused by the inhibition of protein aggregates clearance mediated by autophagy (Le Ber et al., 2013; Shimizu et al., 2013; Teyssou et al., 2013). The gene discussed is SQSTM1; the disease is amyotrophic lateral sclerosis.