TARDBP and amyotrophic lateral sclerosis: The total number of microglia were comparable between normal controls (36 ± 1, n = 3) and ALS cases (36 ± 2, n = 9; p = 0.786); there was a significant decrease of quiescent microglia in ALS patients with TDP-43 pathology (normal controls: 72 ± 1%, n = 3; ALS with TDP-43 pathology: 51 ± 2, n = 9, p < 0.0001; Fig. 1C) and about 21% increase of activated microglia in ALS patients with TDP-43 pathology (normal controls: 28± 1%, n = 3; ALS with TDP-43 pathology: 49 ± 2, n = 9, p < 0.0001; Fig. 1D).