SQSTM1 and amyotrophic lateral sclerosis: In addition, the KEAP1-NRF2 signaling pathway and oxidative response has been shown to be disrupted by SQSTM1 mutations (Deng et al., 2019; Foster et al., 2019), a pathway originally found to be dysregulated in SOD1-ALS (Kirby et al., 2005), which was subsequently implicated in sALS (Sarlette et al., 2008).