Fig. 9: Q84Pfs-Het mice showed myelination deficits.

The immunostaining analyses of Q84Pfs-Het and WT brains with oligodendrocyte lineage cell marker OLIG2 (a, b) and myelination marker MBP (c–e) at P30. Q84Pfs-Het cortex exhibited increased OLIG2+ oligodendrocyte lineage cells, markedly reduced myelinated areas, and disrupted patterns of myelination structure. The number of OLIG2+ cells was measured across three separate regions from the medial to the lateral cortex in coronal sections (b). The proportion of the myelinated area marked by MBP within the total cortical area (d) and the relative fluorescence intensity of MBP in the entire cortical region (e) were quantified. b, d, e n = 6 for WT; 7 mice for Q84Pfs-Het mice. The error bars represent SD. ****p < 0.0001 in unpaired two-tailed t test (b, d, e). Only representative images are shown. Scale bars, 1 mm (the images in the first two columns in a, c), 300 μm (the images in the third column in c), 500 μm (the images in the third column in c), or 100 μm (higher magnification images in a, c). The dotted rectangles marked the area that was magnified in the following columns.