Lines of evidence have indicated that the formation of aggregates is a common feature due to the misfolding of mutant SOD1 in over 100 SOD1 mutants84, and that mutant SOD1 is conjugated to a multi-ubiquitin chain and degraded in a chaperone-dependent manner, which suggests that UPS may play a critical role in the pathological mechanism of ALS. The gene discussed is SOD1; the disease is amyotrophic lateral sclerosis.