[Prion diseases: what is the role of dendritic cells in the pathogenesis of transmissible prion diseases?].
Prion diseases are caused by the transconformation of a normal cellular protein, PrPc, into an infectious isoform, PrPsc, which ultimately triggers neuronal death. They are always fatal and, after transmission, they feature long incubation periods, during which prions accumulate in lymphoid tissues, infect nerves and progress to the central nervous system. In lymphoid organs, prions replicate and accumulate in follicular dendritic cells. Suppressing these cells slows down the neuro-invasion but does not totally abrogate it. This review examines the current knowledge in the roles of hematopoietic dendritic cells at different steps of the pathogenesis of prion diseases. Dendritic cells endocytose inoculated prions, permit their crossing of the intestinal epithelium and then migrate and transport them to lymphoid organs. They can carry prions to sites of neuroinvasion, and establish contacts with axons in peripheral lymph nodes or even after passage of the blood-brain barrier. However, results in the literature on the role of dendritic cells differ according to the host or the prion strain.
Bachy
Véronique
V
Université Pierre et Marie Curie, Paris, France.
Aucouturier
Pierre
P
D000818:Animals; D003713:Dendritic Cells / Q000276:immunology*; D004195:Disease Models, Animal; D006801:Humans; D007410:Intestinal Diseases / Q000276:immunology; D051379:Mice; D017096:Prion Diseases / Q000276:immunology* / Q000635:transmission*; D011328:Prions / Q000502:physiology; D013997:Time Factors
D011328:Prions