ALS is characterized by the progressive degeneration of motor neurons in the brain and spinal cord associated with the accumulation of misfolded proteins and insoluble inclusions.173,174 This protein misfolding disorder can be divided into sporadic and familial ALS.173 The most common genetic form of familial ALS is due to mutations in the SOD1 because they tend to be misfolded and form protease-resistant aggregates.175,176. The gene discussed is SOD1; the disease is amyotrophic lateral sclerosis.