Cellular pathogenesis in prion diseasesCarole Crozet, Florence Beranger and Sylvain Lehmann
Institut de Génétique Humaine, CNRS-UPR1142, 141 rue de la Cardonille, 34396 Montpellier Cedex 5, France
Received 21 September 2007; accepted 15 April 2008 ; published online 17 April 2008
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 prion protein, named PrP. In this review on the “cellular pathogenesis in prion diseases”, we have chosen to highlight the main mechanisms underlying the impact of PrP/PrP on neurons: the neuronal dysfunction, the neuronal cell death and its relation with PrP accumulation, as well as the role of PrP in the microglial and astrocytic reaction.
Key words: Prion / neurodegeneration / cell death / neuronal dysfunction / gliosis
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© INRA, EDP Sciences 2008