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Fig. 2 | Acta Neuropathologica Communications

Fig. 2

From: Neuron loss and degeneration in the progression of TDP-43 in frontotemporal lobar degeneration

Fig. 2

FTLD-TDP cerebral cortex is marked by three tissue grouping denoted by differences in the burden of pTDP-43 inclusions and NeuN positive neuronal nuclei stained by IHC. Progression of FTLD-TDP implicates three Groups of the state of cerebral cortex tissues shown by a IHC in representative images. In Group 1, neuron health is maintained as pathologic pTDP-43 begins to aggregate. Group 2 indicates the peak aggregation of pTDP-43 inclusions. In Group 3, pTDP-43 inclusions and healthy neurons simultaneously lose their immunoreactivity or disappear. Based on our data, we infer that the three Groups represent the sequential stages in the progression of FTDL-TDP in the cerebral cortex regions studies here. Evidence of neurodegeneration increases from Group 1 to Group 3 which is end stage FTLD-TDP disease b Quantification of the tissue in each Group indicates increase in pTDP-43 inclusions in Group 2 (p < 0.001) compared to Group 1, as well as a loss of pTDP-43 pathology in Group 3 compared to Group 2 (p < 0.001). NeuN quantification notes a loss of antigenicity in Group 3 when compared to Group 1 (p < 0.001) and Group 2 (p < 0.001). A Kruskal-Wallis test (p < 0.0001 and p < 0.0001, respectively) followed by Dunn’s test are used to assess significance for both pTDP-43 inclusions and NeuN nuclear staining. The categorization of each tissue section is noted in the scatter plot in (c). Axes are expressed in counts/mm2. For Group 1, n = 87; Group 2, n = 80; and Group 3, n = 106. Bar = 100 μL

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