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. 2022 Oct 11;30(3):716–730. doi: 10.1038/s41418-022-01074-0

Fig. 6. C9orf72 deficiency exacerbates motor deficits and neurodegeneration in poly-GR expression mice.

Fig. 6

a Reduced body weight of C9orf72−/−, AAV-GR50 mice at 6 months of age. Data are mean (n = 6–8 mice; unpaired t-test; *, p < 0.05). b Reduced grip strength of four limbs in C9orf72−/− mice injected with AAV-GR50 at 6 months of age. Data are mean (n = 6–8 mice; unpaired t-test; *, p < 0.05). c Impaired motor performance on an accelerating rotarod test of C9orf72−/−, AAV-GR50 mice at 6 months of age. Data are mean (n = 6–8 mice; unpaired t-test; **, p < 0.01). d Decreased number of NeuN-positive cortical neurons in the motor cortex of C9orf72−/−, AAV-GR50 mice at 6 months of age. The brain slices from mice of indicated genotypes were stained with anti-NeuN antibody to label cortical neurons in the motor cortex. Data are mean (n = 10–13 sections of 3 mice; unpaired t-test; ***, p < 0.001). e Decreased number of ChAT-positive motor neurons in the spinal cord of C9orf72−/−, AAV-GR50 mice at 6 months of age. The lumbar spinal cord slices from mice of indicated genotypes were stained with anti-ChAT antibody to reveal motor neurons in the anterior horn. Data are mean (n = 9–17 sections of 3 mice; unpaired t-test; **, p < 0.01). f Increased neuromuscular junction denervation in C9orf72−/−, AAV-GR50 mice at 6 months of age. The tibialis anterior muscle fibers from mice of indicated genotypes were whole mount stained with CF555-BTX (red) to label AChR clusters and anti-NF/synapsin-1 antibodies (green) to label motor nerve terminals. Arrows, partially denervated NMJ; arrow heads, completely denervated NMJ (n = 9–21 images of 3 mice).