In contrast to human tissue and cultured cells, it has been demonstrated that Nrf2 activity is consistently elevated in the spinal cord of SOD1G93A rodent models of ALS, and the increase of Nrf2, thioredoxin, HSP-70, HO1, NQO1, GCLC, and GCLM protein levels follows disease progression in the lumbar spinal cord but not cortex [264, 265]. Here, NFE2L2 is linked to amyotrophic lateral sclerosis.