Misfolded SOD1 in the form of granular aggregates was regularly detected in the nuclei of ventral horn glial cells including oligodendrocytes in ALS patients carrying or lacking SOD1 mutations [101] as well as in oligodendrocyte cultures derived from fALS or sALS patients presenting mutations in the SOD1 or TDP-43 genes [102]. Here, SOD1 is linked to amyotrophic lateral sclerosis.