We have previously reported that over 100 types of ALS-related SOD1mut but not wild-type SOD1 (SOD1WT) commonly expose the Derlin-1-binding region (termed the DBR) and interact via the DBR with Derlin-1, one of the critical components of the ER-associated degradation (ERAD) machinery13,14. Here, SOD1 is linked to amyotrophic lateral sclerosis.