Fig. 10
RNS60 does not affect the disease progression and the pathology in 129Sv-SOD1G93A mice. Treatment with RNS60 or NS (300 μl/mouse/IP) every other day started from the onset (91 days, hind limbs tremor and reduced abduction) until the symptomatic phase (112 days). a Kaplan-Meier curve shows that the treatment with RNS60 does not modify the onset of muscle strength impairment in fast progressive mice. The curve was evaluated by the Log-rank test, p = 0.1886 (n = 10 NS; n = 10 RNS60). b Quantification of motor neurons with cell body area > 400 μm2 in LSC. At symptomatic stage of the disease, MNs decreased in both SOD1G93A groups of mice compared to NTG without any difference between the treatments. The bar graphs represent mean ± SEM as percentage versus NTG controls (n = 5 animals per group). c Quantification of NMJ denervation in TAM. In transgenic mice there is a higher percentage of denervated plaques compared to NTG. The treatment with RNS60 does not modify these numbers. Data are expressed as mean ± SEM, (n = 5 animals per group). d Quantification of immunoreactivity showed an increase of CD68+ cells in transgenic mice compared to NTG without any change by RNS60 treatment. Data are expressed as mean ± SEM, (n = 5 animals per group). e Quantification of immunofluorescence showed elevated astrocytosis in the LSC ventral horn of 129Sv-SOD1G93A mice at the symptomatic stage of the pathology as compared to NTG expressed as 100%. Treatment with RNS60 significantly decreases the reactive astrocytosis (p < 0.001 SOD1G93A RNS60 vs SOD1G93A NS). f CD68 immunoreactivity quantification showed an up-regulation in the LSC of SOD1G93A mice compared to NTG. This effect was unchanged by the treatment with RNS60. Bar graphs are mean ± SEM as percentage versus NTG (n = 5 animals for each group). All data were statistically analyzed using one-way ANOVA followed by post hoc Fisher’s LSD. *p < 0.05, **p < 0.01, ***p < 0.001, n.s. = non-significant