Fig. 5
From: Motor dysfunction and neurodegeneration in a C9orf72 mouse line expressing poly-PR

Expression of GFP-PR28 causes motor-related neurodegeneration. a Representative images showing the size of the cerebellum and the thickness of the molecular layer of control and GFP-PR28 heterozygous mice at 6 months of age. Neu-N (red), Hoechst (blue). White squares indicates enlarged area. Green lines indicate the thickness of the molecular layer. Scale bar represents 100 μm. b Quantification of the thickness of molecular layer of control and GFP-PR28 heterozygous mice at 2, 6, and 12 months of age. Two months, n = 5, 5 mice; 6 months, n = 5, 5 mice; 12 months, n = 4, 6 mice. c Representative images showing the numbers of cerebellar Purkinje cells of 6-month-old control and GFP-PR28 heterozygous mice. Calbindin (red), Hoechst (blue). Scale bar represents 50 μm. d Quantification of the numbers of calbindin positive Purkinje cells of control and GFP-PR28 heterozygous mice at 2, 6 and 12 months of age. Two months, n = 5, 5 mice; 6 months, n = 5, 5 mice; 12 months, n = 4, 5 mice. e Representative images showing the thickness of motor cortex of control and GFP-PR28 heterozygous mice at 6 months of age. Scale bar represents 100 μm. f Quantification of the thickness of the motor cortex of control and GFP-PR28 heterozygous mice at 2, 6, and 12 months of age. Two months, n = 5, 5 mice; 6 months, n = 5, 4 mice; 12 months, n = 4, 5 mice. g Representative images showing the numbers of ChAT-positive motor neurons in the lumbar spinal cord of 6-month-old control and GFP-PR28 heterozygous mice. GM (gray matter), WM (white matter). Scale bar represents 100 μm. h Quantification of the numbers of ChAT-positive motor neurons of control and GFP-PR28 heterozygous mice at 2 and 6 months of age. Two months, n = 3, 3 mice; 6 months, n = 5, 5 mice. All data aredisplayed as mean ± s.e.m. Two-way ANOVA, Bonferroni post hoc test; *P < 0.05, **P < 0.01, ***P < 0.001, ****P < 0.0001, n.s. not significant