Such a high-expressing hSOD1(G93A) mouse model soon became the most employed animal paradigm for the study of ALS and—in spite of some drawbacks, including the fact that disease severity was rather variable over time and from lab-to-lab (possibly reflecting transgene copy number variations over repeated breeding), as well as the fact that treatments successfully alleviating disease phenotypes in these mice failed in clinical trials [55,56]—it is still widely diffused in research and accepted by most of the ALS scientific community nowadays. Here, SOD1 is linked to amyotrophic lateral sclerosis.