Notably, in vivo studies demonstrate the prion-like propagation of SOD1 aggregates initiated by injection of either aggregated recombinant mutant SOD1 or SOD1WT [12, 13] or homogenates from ALS SOD1G85R or SOD1G93A mice [14] or ALS patients [12] into ALS SOD1G85R mice. Here, SOD1 is linked to amyotrophic lateral sclerosis.