To solve these issues, standard operation procedures (SOPs) for preclinical animal studies for ALS/MNDs has recently been proposed [47], in which in addition to the minimum experimental requirements for any proof of concept or preclinical study, the use of not only SOD1G93A model but also other potential ALS models such as transgenic mice expressing either mutant dynactin, TAR DNA-binding protein (TDP43), or fused in sarcoma (FUS) mutants are recommended. This evidence concerns the gene TARDBP and amyotrophic lateral sclerosis.