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Fig. 5 | Acta Neuropathologica Communications

Fig. 5

From: A robust TDP-43 knock-in mouse model of ALS

Fig. 5

Comparative analysis of patho-signatures and neurodegeneration of cultured spinal cord MN derived from +/+ and mutant ESC. a Survival curves of MN in culture. The percentage(s) of GFP (+) cells in the cell mixtures on day 1 of MN culture is defined as 1. The data showed that (N390D/+) MN became more vulnerable to death than (A315T/+) MN or (+/+) MN on day 14 in culture. Mean ± SD *p < 0.05. b Immunofluorescence co-staining analysis of MN on day 7 and day 14 in culture using anti-ChAT (green or red) and anti-TDP-43 (red or white). The white boxes in the panels of the first column mark the areas magnified for higher resolution. The axonal morphology under lower magnified field is displayed with Z-axis projection. The scale bars are 50 μm. c The statistical analysis of the axon lengths from the WT and mutant MN on different days in culture is shown in the line graph. N > 50. *p < 0.05. d Comparison of the levels of TDP-43 at early stages of ESC-derived MN in culture (from day 1 to day 3), as analyzed by Western blotting. The statistical analysis is shown in the lower scatter plot (mean ± SD). * p < 0.05. e The stability of TDP-43 polypeptides in spinal cord (N390D/+) MN, (A315T/+) MN, and (+/+) MN on day 2 in culture by cycloheximide (CHX) assay and Western blotting analysis. The graph below the blots shows that TDP-43 is more stable in (N390D/+) MN in comparison to either (+/+) MN or to (A315T/+) MN. *p < 0.05

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