In addition to the prominent role of SOD1 in familial ALS, several components of the cellular oxidative stress response have been genetically linked to ALS-FTLD including variants in NFE2L2 and KEAP1, encoding Nrf2 and Keap1 proteins respectively (Bergstrom et al., 2014). Here, KEAP1 is linked to amyotrophic lateral sclerosis.