SQSTM1 and amyotrophic lateral sclerosis: Mitsui et al. (2018) previously reported that loss of SQSTM1 exacerbates disease phenotypes in SOD1H46R ALS mice. Following the initial report, the same authors demonstrated that SQSTM1 overexpression results in a significant increase in biochemically detectable insoluble SQSTM1 and poly-ubiquitinated proteins in the spinal cord of SQSTM1; SOD1H46R mice when compared to SOD1H46R mice. This observation suggests that overexpression of p62 in SOD1H46R mice accelerates disease onset by impairing the protein degradation pathways (Mitsui et al., 2018).