Fig. 2
Kaempferol promotes motor neuron survival and reduces pathology progression. a Immunohistochemical staining for MN marker ChAT and quantitative analyses. reveals significant differences in motor neuron numbers between C9-500 mice treated with KMP until the end stage of the disease at P240 and the saline group (Unpaired t-test: C9-500 + saline mean ± SEM 21.75 ± 1.097 vs C9-500 + KMP mean ± SEM 32.48 ± 1.127, t = 6.455, P < 0.0001). Scale bar = 150 μm. b ChAT-positive C9-500 MNs from C9-500 mice show in an age-dependent manner the accumulation of greater than 1µm3 poly-GA aggregates. (MN numbers: P60 C9-500: n = 42; P125 C9-500: n = 47; P150 C9-500: n = 38; from 4 mice/genotype/age). Scale bar: 30 μm. c Quantitative analyses of Poly(GA) aggregates reveal a significant reduction in the number of aggregates in C9-500 mice treated with KMP (Unpaired t-test: C9-500 + saline: n = 19 MNs vs C9-500 + KMP; n = 22 MNs, t = 3.110, P = 0.0035**). Quantitative analysis of PolyGA aggregate volume reveals a significant decrease in > 1 μm3 aggregates within spinal MN after KMP treatment (Unpaired t-test: C9-500 + saline: n = 19 MNs vs C9-500 + KMP; n = 22 MNs, t = 3.632, P = 0.0011**). n = 4–5 mice/genotype/treatment. Scale bar: 10 μm. d Representative confocal images of GFAP staining and quantitative analysis. Note the reduced astrogliosis in C9-500 animals treated with KMP (Unpaired t-test: C9-500 + saline vs C9-500 + KMP (t = 5.789, P < 0.0001***). e Representative images of muscle sections with staining for H&E from C9-500 mice treated with saline and KMP. Blue arrowheads in the H&E images point to small, and degenerating fibers that are not present in C9-500 mice treated with KMP. Scale bar: 100 μm. f Representative images of muscle sections with staining NADH from C9-500 mice treated with saline and KMP. Red arrows in the NADH staining point to small and degenerating fibers that are not present in C9-500 mice treated with KMP. Moreover, the NADH staining presents pale fibres in C9-500 saline animals while in KMP-treated C9-500 mice, a strong checkboard pattern is present. Scale bar: 150 μm