Cellular pathogenesis in prion diseases.
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.
Crozet
Carole
C
Institut de Génétique Humaine, CNRS-UPR1142, 34396 Montpellier Cedex 5, France.
Beranger
Florence
F
Lehmann
Sylvain
S
D016923:Cell Death; D009410:Nerve Degeneration; D009474:Neurons / Q000166:cytology*; D018620:PrPSc Proteins / Q000378:metabolism*; D017096:Prion Diseases / Q000473:pathology*; D011328:Prions / Q000378:metabolism
D018620:PrPSc Proteins; D011328:Prions