Cellular pathogenesis in prion diseases

Carole Crozet , Florence Beranger , Sylvain Lehmann

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$^{\rm Sc}$. In this review on the "cellular pathogenesis in prion diseases", we have chosen to highlight the main mechanisms underlying the impact of PrP$^{\rm C}$/PrP$^{\rm Sc}$ on neurons: the neuronal dysfunction, the neuronal cell death and its relation with PrP$^{\rm Sc}$ accumulation, as well as the role of PrP$^{\rm Sc}$ in the microglial and astrocytic reaction.

Mots clés: Prion; neurodegeneration; cell death; neuronal dysfunction; gliosis

Domaines: Biologie moléculaire; Génétique animale; Biologie cellulaire; Interactions cellulaires [q-bio.CB]; Microbiologie et Parasitologie; Immunologie; Neurosciences [q-bio.NC]; Santé publique et épidémiologie; Biologie animale

Carole Crozet, Florence Beranger, Sylvain Lehmann. Cellular pathogenesis in prion diseases. Veterinary Research, 2008, 39 (4), pp.1-17. ⟨10.1051/vetres:2008021⟩. ⟨hal-00902924⟩