We have also shown that ectopic expression of PDI, one of the chaperone proteins, translocated the SOD1G93A into the nucleus, rescued its cytosolic mislocalization, and protected neuronal death under high ER stress, suggesting the possible connection between SOD1G93A mislocalization and neuronal death in SOD1 mutation-linked ALS. This evidence concerns the gene SOD1 and amyotrophic lateral sclerosis.