Our endogenous KI model is thus novel and distinct from previous ALS-TDP-43 overexpression and downregulation mice models [27, 45, 46, 66–68], and NLS-deleted Tdp-43 variant from the murine Tardbp gene’s locus is expressed, specifically in MNs. The gene discussed is PSME3; the disease is amyotrophic lateral sclerosis.