Fig. 7
Inhibition of NF-κB with IMS-088 attenuates age-dependent pathological features in hFUSR521G/Syn1 mice. a The spinal cord of mice co-stained with anti-acetylated P65 (acP65) and anti-NeuN show that IMS-088 blocks the activation and nuclear localisation of acP65 in neurons and non-neuronal cells of treated hFUSR521G/Syn1 mice. b Spinal cord staining with anti-GFAP and anti-Iba1 in mice. c Signal intensity (S.I) for GFAP and Iba1 shows IMS-088 inhibits activation of astrocytes and microglial in treated FUS transgenic mice compared to vehicle treated mice. d Spinal cord staining with anti-hFUS, anti-NeuN and DAPI show IMS-088 restores nuclear distribution of hFUS in hFUSR521G/Syn1 mice. e Spinal cord staining with anti-TOM20. Quantification of TOM20 shows that IMS-088 restores f S.I. and g number of puncta, area and volume in 6-months-old FUS transgenic mice. Values from each group are expressed as mean ± SEM. Statistics uses a one-way ANOVA for multiple group comparisons (n = 3–4 mice/group). *p < 0.05, **p < 0.01, ***p < 0.005, ****p < 0.001, and not significant (ns)