Additionally, autophagy is the principal intracellular catabolic route for removing misfolded proteins, aggregates, and damaged organelles that cause aging and neurodegeneration like ALS, in which autophagy is frequently disrupted, leading to cytoplasmic separation of the readily aggregated and toxic proteins in neurons, especially dysfunctional SOD1 to produce ROS. The gene discussed is SOD1; the disease is amyotrophic lateral sclerosis.