Fig. 1

Expression of TDP-43^WT and TDP-43^Q331K in mice decreases endogenous TDP-43 expression. a Western blotting of brain lysate from 8 week old non-transgenic (NTg), TDP-43^WT, TDP-43^Q331K, and TDP-43^WTxQ331K mice using an anti-TDP antibody showed a slight shift to a higher molecular weight due to the presence of the myc tag. There was an increase in total TDP43 expression in all transgenic animals, with higher expression in TDP-43^Q331K mice than TDP-43^WT animals, and TDP-43^WTxQ331K animals showing evidence of cumulative TDP43 expression (b). This increase was accompanied by a concomitant decrease in endogenous TDP43 expression (c). d–k Immunohistochemistry with anti-myc antibody showed expression of the TDP-43 protein throughout the spinal cord (d–g) and in the cortex (h–k; scale bar: 50 μm) in TDP^WT (e, i), TDP^Q331K (f, j) and TDP-43^WTxQ331K (g, k) mice, which was absent in NTg animals (d, h) . (All graphs are mean ± SEM; * p < 0.05; ** p < 0.001 vs. NTg; †† p < 0.001 vs. TDP^WT, ## p < 0.001 vs. TDP^Q331K)