Fig. 4
Neuron-specific expression of FUSR521G promotes age-dependent neuropathological features of ALS/FTD. The brain and spinal cord of a 1-month-old and c 6-months-old CTL and hFUSR521G/Syn1 mice stained with anti-GFAP (astrocyte marker) and anti-Iba1 (microglia marker). Signal intensity (S.I) for GFAP and Iba1 show b no significant changes in 1-month-old hFUSR521G/Syn1 mice and d significant activation of astrocytes and microglia in 6-months-old FUS transgenic mice compared to CTL mice. e, f Spinal cord staining with anti-hFUS, anti-NeuN and DAPI show mislocalisation of hFUS in 6-months-old hFUSR521G/Syn1 mice. g Spinal cord staining with the mitochondrial marker, TOM20. Quantification of TOM20 shows a significant decrease in h S.I. and i number of puncta, area and volume in 6-months-old hFUSR521G/Syn1 mice. Values from each group are expressed as mean ± SEM. Statistics uses an unpaired Student’s t-test for comparison between two groups and a one-way ANOVA for multiple group comparisons (n = 3–4 mice/group). **p < 0.01, ***p < 0.005 ****p < 0.001, and not significant (ns)