The above-described similar phenotypes reproduced by the mutant TDP-43 transgenic C. elegans models reproduce major features seen in ALS patients, such as progressive muscle weakness and atrophy, shortened life span, and MNs degeneration accompanied by both hyperphosphorylation, truncation, and ubiquitination of TDP-43 proteins and the accumulation of detergent-insoluble TDP-43 proteins. Here, TARDBP is linked to amyotrophic lateral sclerosis.