Fig. 7

Model of targeting Bergmann glia activation to combat SCAs. Top panel SCA patients and mouse cerebellums exhibit Bergmann glia (BG)-specific JNK-dependent c-Jun phosphorylation (black nuclei); the time when BG are known to have a reactive state is marked by enhanced GFAP intensity (red processes). These reactive BG release the enhanced proinflammatory cytokine IL-1β into the cerebellum in a JNK-dependent manner. Bottom panel Treatment of SCA1 mice with JNK inhibitor SP600125 abolishes the c-Jun phosphorylation in BG (light gray nuclei) and thereby tamps down the reactive GFAP staining and cytokine IL-1β release in the cerebellum. These changes in inflammation lead to a decrease in Purkinje cell pathology (green) and a rescue of motor deficits