title: Cellular pathogenesis in prion diseases.
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(Sc). In this review on the "cellular pathogenesis in prion diseases", we have chosen to highlight the main mechanisms underlying the impact of PrP(C)/PrP(Sc) on neurons: the neuronal dysfunction, the neuronal cell death and its relation with PrP(Sc) accumulation, as well as the role of PrP(Sc) in the microglial and astrocytic reaction.
authors.affiliation: Institut de Génétique Humaine, CNRS-UPR1142, 34396 Montpellier Cedex 5, France.
authors.forename: Carole
authors.initials: C
authors.lastname: Crozet
authors.forename: Florence
authors.initials: F
authors.lastname: Beranger
authors.forename: Sylvain
authors.initials: S
authors.lastname: Lehmann
mesh_terms: D016923:Cell Death; D009410:Nerve Degeneration; D009474:Neurons / Q000166:cytology*; D018620:PrPSc Proteins / Q000378:metabolism*; D017096:Prion Diseases / Q000473:pathology*; D011328:Prions / Q000378:metabolism
chemical_list: D018620:PrPSc Proteins; D011328:Prions