As a consequence, the Fe2+-bound hSOD1 form without the disulfide bridge would ultimately acquire high toxicity as found with other ALS-causing mutants including G93A47 and H46R/H48Q48, by becoming aggregated to form the iron-containing hSOD1 inclusion (Fig. 10H) which has been extensively observed in ALS patients. Here, SOD1 is linked to amyotrophic lateral sclerosis.