Research Paper Volume 8, Issue 11 pp 2667—2688

The progeroid gene BubR1 regulates axon myelination and motor function

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Figure 4. BubR1 insufficiency causes defects in CNS myelination in 8-week-old BubR1 insufficient mice. (A-B) Luxol fast blue (LFB) staining analysis. (A) Sample images of LFB staining in WT and BubR1H/H mice (left) and quantification of LFB area in coronal brain sections of 8-week-old mice (right). BubR1H/H mice exhibit a profound reduction in myelin density in the corpus callosum (blue dashed box), and internal capsule (red dashed box). Scale bar: 0.2 cm. (B) Sample images (left) and quantification of LFB area (right) indicating a profound reduction in myelin density in the spinal cord of BubR1H/H mice. Scale bar: 500 µm. WM; white matter, GM; gray matter. (C-G) Electron microscopy (EM) imaging analysis of the spinal cord dorsal column white matter of BubR1H/H mice and their WT littermates. Hypomyelination is observed in the spinal cord of BubR1H/H mice. (C) Representative EM images are shown. Scale bars: 2 µm for low magnification, and 0.2 µm for high magnification. (D,E) Scatter diagram and quantification of G-ratio. (F,G) Scatter diagram and quantification of myelin thickness. All values represent mean ± SEM (**P < 0.01, ***P < 0.001, student’s t-test). Number associated with bar graphs indicates number of animals examined.