Abstract
Prion diseases are characterised by neuronal loss, vacuolation (spongiosis), reactive astrocytosis,microgliosis and in most cases by the accumulation in the central nervous system of the abnormal prionprotein, named PrPSc. In this review on the “cellular pathogenesis in prion diseases”, we have chosen tohighlight the main mechanisms underlying the impact of PrPC/PrPScon neurons: the neuronal dysfunction,the neuronal cell death and its relation with PrPScaccumulation, as well as the role of PrPScin the microglialand astrocytic reaction.