In both ALS patients and mouse models, SpMNs and astrocytes typically contain inclusions positive for ubiquitinated proteins and the ubiquitin-binding protein SQSTM1 (Sequestosome 1, also known as p62) (Leigh et al., 1991; Watanabe et al., 2001; Mizuno et al., 2006; Neumann et al., 2006; Gal et al., 2007). This evidence concerns the gene SQSTM1 and amyotrophic lateral sclerosis.