Free Access
Vet. Res.
Volume 39, Number 4, July-August 2008
Prion diseases in animals
Number of page(s) 17
Published online 17 April 2008
How to cite this article Vet. Res. (2008) 39:44
How to cite this article: Vet. Res. (2008) 39:44
DOI: 10.1051/vetres:2008021


Cellular pathogenesis in prion diseases

Carole 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$^{\rm Sc}$. 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.

Key words: Prion / neurodegeneration / cell death / neuronal dysfunction / gliosis

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© INRA, EDP Sciences 2008