NFE2L2 and amyotrophic lateral sclerosis: The administration of CDDO-EA and CDDO-TFEA at a presymptomatic age enhanced motor performance and extended the survival of SOD1G93A mice, while at a symptomatic age, it only slowed disease progression [178] (Table 2), suggesting that the activation of the Nrf2/ARE signaling pathway may be a useful strategy in the treatment of ALS especially when administered early in the course of the disease.