In contrast, SOD1-positive inclusions with ALS-like symptoms were reproduced in a fALS-model mouse expressing human SOD1 with a pathogenic mutation (Turner and Talbot, 2008) and were found to be stained by Thioflavin S, supporting the formation of amyloid-like, β-sheet-rich fibrils in mouse (Wang et al., 2002; Furukawa et al., 2008). Here, SOD1 is linked to amyotrophic lateral sclerosis.