Nrf2 expression was found to be decreased in NSC-34 cells expressing mutSOD1, in MNs isolated from familial SOD1-associated ALS patients [110], and in primary motor cortex and spinal cord postmortem tissue samples from ALS patients [111], which suggested that increasing neuronal Nrf2 activity may represent a novel therapeutic target. Here, NFE2L2 is linked to amyotrophic lateral sclerosis.