Over the last 20 years, several transgenic mouse strains expressing human mutant SOD1 have been generated. These mice have been used to either examine disease mechanisms or trial potential therapeutic strategies for ALS, although the latter has led to questionable success (Perrin, 2014) (Tables 5, 6). The transgenic line harboring the Gly93 → Ala substitution (SOD1G93A) has been used most extensively (Gurney et al., 1994), followed by the SOD1G37R (Wong et al., 1995), SOD1G85R (Bruijn et al., 1997), SOD1G86R (Ripps et al., 1995) and SOD1D90A (Jonsson et al., 2006) models.