Protein aggregates positive for TDP-43 [36,205], neurofilament [41], FUS [87], or SOD1 [206] are observed in the vast majority of ALS patients, with TDP-43 being present in as many as 98% of sporadic and familial cases [207], meaning that the presence of such aggregates is widely regarded as a hallmark feature of ALS pathology. The gene discussed is SOD1; the disease is amyotrophic lateral sclerosis.