Early microglial activation precedes neuronal loss in the brain of the Cstb-/- mouse model of progressive myoclonus epilepsy, EPM1.

@article{Tegelberg2012EarlyMA,
  title={Early microglial activation precedes neuronal loss in the brain of the Cstb-/- mouse model of progressive myoclonus epilepsy, EPM1.},
  author={Saara Tegelberg and Outi H Kopra and Tarja Joensuu and Jonathan D Cooper and A E Lehesjoki},
  journal={Journal of neuropathology and experimental neurology},
  year={2012},
  volume={71 1},
  pages={40-53}
}
Progressive myoclonus epilepsy of Unverricht-Lundborg type (EPM1) is a hereditary neurodegenerative disorder caused by mutations in the cystatin B (CSTB) gene encoding an inhibitor of cysteine proteases. Here, we provide the first detailed description of the onset and progression of pathologic changes in the CNS of Cstb-deficient (Cstb) mice. Our data reveal early and localized glial activation in brain regions where neuron loss subsequently occurs. These changes are most pronounced in the… CONTINUE READING
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