Figure 2.

Progressive loss of UMN is confirmed by in situ hybridization. (A) Representative negative fluorescence images of brain coronal sections (Bregma 0.74 mm, corresponding to Section 5 for Figure 1) showing FG-labelled UMN in the cerebral cortex of WT and Sod1^G86R mice of 60, 75, 90, and 105 days of age or end stage (ES). Red shapes indicate the positions where the close-ups of the right panels were acquired. (B) Representative images of in situ hybridization on brain coronal sections of ES Sod1^G86R and 115 day-old WT control mice showing decreased Crym expression in the layer V of the cerebral cortex. Dotted red rectangles indicate the areas where Crym-positive neurons were counted. Close-ups are shown in the right panel. (C) Bar graph representing the percentage of FG-positive neurons in Sod1^G86R mice (orange) relative to their WT littermates (blue) over time. (D) Bar graph representing the percentage of Crym-positive neurons in Sod1^G86R mice (orange) relative to their WT littermates (blue) over time. N = 5 WT and 6 Sod1^G86R at 60 days; 6 WT and 3 Sod1^G86R at 75 days; 5 WT and 6 Sod1^G86R at 90 days; 5 WT and 5 Sod1^G86R at 105 days/ES (end stage). ** p < 0.01 and *** p < 0.001 in multiple comparisons test. Scale bars = 200 µm in the left panels and 50 µm in the right panels of (A,B). M1, primary motor cortex; M2, secondary motor cortex; S1, primary somatosensory cortex.