Collectively, these findings suggest the ataxia observed in Smox/Sat1-dKO mice is initiated as a result of Purkinje cell damage and gliosis (neurodegenerative M1 microglia and A1 astrocyte activation), which is later exacerbated by white matter demyelination and the onset of a reactive immune/inflammatory response (e.g., CD4+ and CD8+ T cell leptomeningeal infiltrates). The gene discussed is SAT1; the disease is Ataxia.