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Open Access 01-12-2016 | Short report

Brain inflammation is accompanied by peripheral inflammation in Cstb ^−/− mice, a model for progressive myoclonus epilepsy

Authors: Olesya Okuneva, Zhilin Li, Inken Körber, Saara Tegelberg, Tarja Joensuu, Li Tian, Anna-Elina Lehesjoki

Published in: Journal of Neuroinflammation | Issue 1/2016

Abstract

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 brains. The elevated chemokine levels were not accompanied by blood-brain barrier disruption, despite increased brain vascularization. Macrophages in the spleen and brain of Cstb ^−/− mice were predominantly pro-inflammatory. Taken together, these data show that CXCL13 expression is a hallmark of microglial activation in Cstb ^−/− mice and that the brain inflammation is linked to peripheral inflammatory changes, which might contribute to the disease pathology of EPM1.

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Title: Brain inflammation is accompanied by peripheral inflammation in Cstb −/− mice, a model for progressive myoclonus epilepsy
Authors: Olesya Okuneva
Zhilin Li
Inken Körber
Saara Tegelberg
Tarja Joensuu
Li Tian
Anna-Elina Lehesjoki
Publication date: 01-12-2016
Publisher: BioMed Central
Published in: Journal of Neuroinflammation / Issue 1/2016
Electronic ISSN: 1742-2094
DOI: https://doi.org/10.1186/s12974-016-0764-7

At a glance: The STEP trials

Springer Medicine

Brain inflammation is accompanied by peripheral inflammation in Cstb ^−/− mice, a model for progressive myoclonus epilepsy

Abstract

At a glance: The STEP trials

Springer Medicine

Abstract

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