SOD1 and amyotrophic lateral sclerosis: Autophagy is a major degradation pathway of misfolded or unfolded proteins as well as the ubiquitin-proteasomal pathway41 and regulation of basal autophagy is crucial for neural survivals.42,43 Impairment of the ubiquitin-proteasomal pathway, which has been reported in both SOD1-related17,18 and SOD1-unrelated19,20 ALS models implies that activation of the autophagy pathway may complementally contribute to degradation of abnormal toxic proteins.