NFE2L2 and amyotrophic lateral sclerosis: However, increased levels of ROS in lymphoblasts from sporadic ALS subjects were able to activate the Nrf2 response by stimulating the expression of downstream proteins, while Nrf2 and NQO1 protein levels were lower in lymphoblasts derived from SOD1-related ALS subjects, suggesting that the Nrf2-mediated antioxidant response was activated in the sporadic ALS model, but not in the model of familial ALS.