Indeed, the first model of ALS, the SOD1G93A transgenic strain [Tg(SOD1*G93A)1Gur], was published a year after the discovery of SOD1-ALS mutations in humans (Gurney et al., 1994) (Table 1A) and remains the most commonly used ALS mouse model. Here, SOD1 is linked to amyotrophic lateral sclerosis.