Figure 3.
Pathological alterations in the brain of 9-month-old Gnptabc.3082insC mice. (A) Purkinje cells in cerebellar sections were stained for calbindin. Higher-magnification images of the regions marked by the black rectangles revealed axonal spheroids of Purkinje cells (arrows) in Gnptabc.3082insC mice. Scale bars: upper panel = 500 µm; insets = 50 µm. (B) Luxol fast blue staining of the cerebellar sections indicates decreased myelination and degeneration of the subcortical white matter in the Gnptabc.3082insC mouse. Scale bars: upper panel = 500 µm; insets = 50 µm. (C) Strong immunoreactivity for glial fibrillary acidic protein (GFAP) throughout the Gnptabc.3082insC brain. Insets represent higher-magnification images of the cortical region marked by the black rectangles. Scale bars: cerebellum = 500 µm; cortex = 250 µm; insets = 50 µm. (D) Activated microglial cells immunoreactive for CD68 are observed in the cerebellum and the hippocampus of Gnptabc.3082insC mice. Insets reveal the activated phagocytic shape of the microglial cells. Scale bars: cerebellum = 500 µm; hippocampus = 500 µm; insets = 50 µm.