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Fig. 3 | Journal of Neuroinflammation

Fig. 3

From: RNS60 exerts therapeutic effects in the SOD1 ALS mouse model through protective glia and peripheral nerve rescue

Fig. 3

RNS60 prevents MN dysfunction, loss and reduces the hSOD1-positive vacuoles in C57BL/6-SOD1G93A mice. a–c Representative images of NISSL staining performed on LSC sections from NTG and C57BL/6-SOD1G93A mice treated with NS (SOD1G93A NS) or RNS60 (SOD1G93A RNS60) at the symptomatic stage of the disease (140 days). Scale bar: 100 μm. d Quantification of motor neurons with cell body area > 400 μm2 in LSC. At symptomatic stage of the disease, MNs were decreased in both transgenic groups of mice compared to NTG. Treatment with RNS60 displayed significant neuroprotection (46.9 ± 2.62%) compared to SOD1G93A mice treated with NS (22.6% ± 1.7). The bar graph represents mean ± SEM as percentage versus NTG controls (n = 3 animals per group). e–g RNS60 reduced the accumulation of phosphorylated neurofilaments (SMI31 green) in the MN cell body (arrow) labeled with neurotrace (NT, red) and proximal axons (arrow head) which is an index of MN dysfunction (scale bar: 20 μm). h In transgenic mice, we observed a marked increase of SMI31-labeled MNs compared to NTG mice (8.9 ± 1.3%). RNS60 significantly reduced the number of impaired MN (30.1 ± 2.4%) compared to NS-treated SOD1G93A mice (43.3 ± 3.7%). The bar graph represents mean ± SEM (n = 3 mice per group). i–k Laser scanning confocal photomicrographs of hSOD1 staining (green) in LSC MNs of NTG and transgenic mice treated with NS or RNS60 (scale bar: 20 μm). l Quantification showed the elevated level of hSOD1 immunoreactivity in transgenic LSC compared to NTG. This effect was significantly reduced by the treatment with RNS60. The bar graph represents mean ± SEM as a percentage versus NTG (n = 3 mice per group). All data were statistically analyzed using one-way ANOVA followed by post hoc Fisher’s LSD. **p < 0.01, ***p < 0.001

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