Fig. 9

Smox/Sat1-dKO mice develop severe demyelination and neuronal injury. a LFB staining reveals the presence of demyelinating injury in the deep cerebellar nuclei of Smox/Sat1-dKO mice at 8 weeks of age and severe white matter demyelination and leptomeningeal leukocytic infiltration in the cerebellum of 14+-week-old Smox/Sat1-dKO mice. b Northern and western blot analyses of cerebellar RNA (top two panels) and protein extracts (bottom two panels) revealed a decrease in the expression of MBP mRNA (top panel) and protein (third panel from the top) levels in the cerebellum of Smox/Sat1-dKO mice. Northern blots contained 25 μg/lane of RNA and western blots contained 50 μg/lane of protein. Results are representative of 3 independent experiments. c Transmission electron microscopic analysis of cerebellar myelin sheaths revealed the onset of progressive age-dependent damage (increased translucency and disorganization of the myelin lamellae) in Smox/Sat1-dKO mice. Results are representative of TEM images obtained from multiple samples; n = 3/genotype/time point. d Severely ataxic mice develop profuse leptomeningeal lymphocyte infiltrates rich in CD4⁺ and CD8⁺ cells