Article
A pathogenetic hypothesis of Unverricht-Lundborg disease onset and progression.
Neurobiology of disease - 1 Mar 2007
Franceschetti Silvana, Sancini Giulio, Buzzi Andrea, Zucchini Silvia, Paradiso Beatrice, Magnaghi Giuseppina, Frassoni Carolina, Chikhladze Maia, Avanzini Giuliano, Simonato Michele
Abstract excerpt
Unverricht-Lundborg disease (EPM1), the most common progressive myoclonic epilepsy, is associated with a defect of cystatin B (CSTB), a protease inhibitor. We used CSTB knockout mice to test the hypothesis that EPM1 onset is related to a latent hyperexcitability and that progression depends on higher susceptibility to seizure-induced cell damage. Hippocampal slices prepared from CSTB-deficient mice were...
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