Preclinical trials using other animal models of the disease should be done in parallel with the common mutSOD1 models, including C9orf72 and TDP-43 mice models that represent the most prevalent mutation in ALS and the formation of ubiquitinated TDP-43 cytoplasmic inclusions that are expressed in the majority of patients, respectively [276, 277]. The gene discussed is TARDBP; the disease is amyotrophic lateral sclerosis.