Fig. 6
Mutation of Ubap1 results in the accumulation of ubiquitinated proteins and loss of neurons in the spinal cord. A Confocal images of spinal cord sections from 7-month-old mice stained with ubiquitin (red), LC3 (green), and DAPI (blue). Yellow in the merged images indicates colocalization of ubiquitin and LC3. Scale bar, 10 μm. B Comparison of the area stained with ubiquitin in a single neuron is shown in the graph (WT: n = 28, KI: n = 30; Mann–Whitney U test; ***: p < 0.001). C, D Quantification of ubiquitinated proteins from mouse spinal cord tissue by Western blotting. The expression of GAPDH is shown as a loading control. Mutations in Ubap1 result in increases in the ubiquitinated proteins (WT: N = 4, KI: N = 4; Welch’s t-test; *: p < 0.05). E Confocal images of spinal cord sections from 7-month-old mice stained for NeuN (green). The image on the right is a higher magnification of the area indicated by the square. Scale bar, 100 μm. F Quantification was performed. Mutations in UBAP1 reduced the number of neurons in the spinal cord (WT: n = 14, KI: n = 11; Mann–Whitney U test; *: p < 0.05). G Mutations in Ubap1 caused an increase in the size of the anterior horn cells of the spinal cord (WT: n = 55, KI: n = 42; Mann–Whitney U test; ***: p < 0.001)
