SOD1 and amyotrophic lateral sclerosis: Supporting this, Kraft et al. (2007) evaluated the endogenous activation of the Nrf2-ARE system during the induction of pathology in mutant SOD mouse models and showed that Nrf2-ARE activation appears to progress in a retrograde fashion along the motor pathway, thereby implying the contributions of the Nrf2-ARE pathway and its activation which can persist throughout ALS pathology and which increases in concert with disease severity [43].