TARDBP and amyotrophic lateral sclerosis: To further validate the MN-Tdp-43∆NLS mouse model of ALS, we performed IHC-IF assays by co-staining with anti-Map2 and Tdp-43 antibodies in the deep cortical layers (III-IV), revealing that 53.5% (P < 0.0001) more Map2-positive neurons predominantly had the cytosolic Tdp-43 mislocalization phenotype in MN-Tdp-43∆NLS mice than sham mice (Fig. 3A-B), along with distorted MN nuclei and reduced expression of Map2 (-1308 ± 113.2 a.u.; P < 0.0001; Supplemental Fig. 1C), suggesting an MND pathology in the brain of MN-Tdp-43∆NLS mice.