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. 2021 Feb 24;12:1265. doi: 10.1038/s41467-021-21021-w

Fig. 4. The effect of ASO–CCG on behavioral and molecular phenotypes in the FXTAS mouse model.

Fig. 4

a Representative image showing the distribution of ASO–CCG-Cy3 (red; 200 nM) in COS7 cells. ASO entered cells in the absence of a carrier. Blue, stained nuclei; scale bar, 20 µm. The experiment was repeated two times with similar results. b Experimental design for DOX induction (red) and ASO–CCG delivery (blue) into P90CGG mice. The contents of the osmotic pump (ASO–CCG (11 nt) solution or saline) were delivered into the right lateral ventricle via intracerebroventricular infusion (~550 µg of ASO–CCG per mouse). c Training session for the rotarod test. Mice treated with saline and ASO–CCG were subjected to a 3-day rotarod test starting with a training session performed at 15 rpm constant speed (day 1). No differences were observed between groups. d Constant speed rotarod test. On day 2, the above-mentioned mice were subjected to a constant speed rotarod test at different speeds (x axis; rpm, revolution per minute). The ASO–CCG-treated group stayed longer on the rod, P = 0.0323. e Accelerating rotarod test. On day 3, mice were subjected to an accelerating rotarod test (4–40 rpm ramp). The ASO–CCG-treated group showed better performance on this test (longer latency to fall), P = 0.0003. Dashed line, maximum cutoff c, d 60 s, e 300 s. f Quantification of FMRpolyG foci in P90CGG mice. A representative image of FMRpolyG staining, sections containing cerebellar lobule X of P90CGG mice was stained using the 8FM antibody specific for FMRpolyG (scale bar, 10 µm). For the ASO–CCG-treated group, the percentage of the nuclei with positive staining for FMRpolyG (red) was lower (P = 0.0161) and the size of the inclusions they contain (indicated by a white arrow) were smaller (P = 0.0146) than that of the saline-treated group. Graphs present means with SDs. a, f Images were pseudo-colored and merged. c, d Two-way repeated-measures ANOVA (treatment effect): *P < 0.05. e, f Two-sided, unpaired Student’s t test: *P < 0.05; ***P < 0.001. c–e Graphs present means with SEMs. c–f Gray dots/bars, saline-treated animals (N = 6); blue squares/bars, ASO–CCG treated animals (N = 7). Source data are provided as a Source Data file.