Genetic activation of Nrf2 protects against neuronal and cognitive decline in Drosophila and mouse models of AD and PD (Kanninen et al., 2009; Barone et al., 2011; Kerr et al., 2017), and pharmacological Nrf2 activators, including triterpenoid compounds (CDDO-EA and CDDO-TFEA) and dimethyl fumarate (DMF), prevent oxidative damage and afford neuronal protection in mouse models of AD (Dumont et al., 2009), FTD (Cuadrado et al., 2018), amyotrophic lateral sclerosis (ALS; Neymotin et al., 2011), PD (Chen et al., 2009; Lastres-Becker et al., 2016) and cerebral ischemia (Fowler et al., 2017). This evidence concerns the gene NFE2L2 and amyotrophic lateral sclerosis.