查看文章

Progressive myoclonus epilepsy of Unverricht-Lundborg type (EPM1) is an autosomal recessively inherited childhood-onset neurodegenerative disorder, characterized by myoclonus, seizures, and ataxia. Mutations in the cystatin B gene (CSTB) underlie EPM1. The CSTB-deficient (Cstb ^−/− ) mouse model recapitulates key features of EPM1, including myoclonic seizures. The mice show early microglial activation that precedes seizure onset and neuronal loss and leads to neuroinflammation. We here characterized the inflammatory phenotype of Cstb ^−/− mice in more detail. We found higher concentrations of chemokines and pro-inflammatory cytokines in the serum of Cstb ^−/− mice and higher CXCL13 expression in activated microglia in Cstb ^−/− compared to control mouse …