SOD1 and amyotrophic lateral sclerosis: Although Bunina bodies are the specific structures in patients with sporadic ALS, they are not found in some sporadic ALS cases as well as cases of familial ALS caused by SOD1 mutations.26,27 In a previous study, CysC was immunostained in remaining neurons as a condensed form and activated astrocytes in SOD1G93A mouse spinal cord.24 Therefore, we first confirmed the localization of endogenous murine CysC and its alternations in SOD1G93A mouse spinal cord (Figure 1).