In conclusion, the identification of ALS-associated genetic defects in SOD1, FUS, TDP-43, C9ORF72, TBK1, and OPTN, and the abnormalities in protein homeostasis and autophagosomes associated with these defects support an important role for dysregulated autophagy in ALS pathogenesis. The gene discussed is OPTN; the disease is amyotrophic lateral sclerosis.