Cystatin B knockout model of progressive myoclonus epilepsy: cultured cerebellar granule cells
Summary:
Analysis of granule cells dissected from postnatal day 5 cystatin B (CSTB)-deficient mice and cultured for 2 days. Progressive myoclonus epilepsy Unverricht-Lundborg type (EPM1) is a neurodegenerative disease caused by CSTB gene mutations. Results provide insight into the molecular basis of EPM1.
Joensuu T, Tegelberg S, Reinmaa E, Segerstråle M et al. Gene expression alterations in the cerebellum and granule neurons of Cstb(-/-) mouse are associated with early synaptic changes and inflammation. PLoS One 2014;9(2):e89321. PMID: 24586687
