Our endogenous KI model is thus novel and distinct from previous ALS-TDP-43 overexpression and downregulation mice models [27, 43, 44, 59-61], and NLS-deleted Tdp-43 variant from the murine Tardbp gene’s locus is expressed, specifically in motor neurons. The gene discussed is TARDBP; the disease is amyotrophic lateral sclerosis.