In particular, these include models of amyotrophic lateral sclerosis, i.e., the SOD-1-G93A and A315T-TDP-43 strains (Jackson Laboratory, Bar Harbor, ME, USA) (Kaspar et al., 2003; Ishiyama et al., 2004; Turner et al., 2009; Wegorzewska et al., 2009; Towne et al., 2010; Xu et al., 2010; Guo et al., 2011; Riboldi et al., 2011). Here, SOD1 is linked to amyotrophic lateral sclerosis.