| Autor: |
Levavasseur E; Institut du Cerveau et de la Moelle épinière, ICM, Inserm U 1127, CNRS UMR 7225, Sorbonne Université, 75013 Paris, France. etienne.levavasseur@icm-institute.org., Privat N; Institut du Cerveau et de la Moelle épinière, ICM, Inserm U 1127, CNRS UMR 7225, Sorbonne Université, 75013 Paris, France. nicolas.privat@inserm.fr., Haïk S; Institut du Cerveau et de la Moelle épinière, ICM, Inserm U 1127, CNRS UMR 7225, Sorbonne Université, 75013 Paris, France. stephane.haik@upmc.fr.; Assistance Publique-Hôpitaux de Paris, Cellule nationale de référence des MCJ, G.H. Pitié-Salpêtrière, 75013 Paris, France, AP-HP, Laboratoire de Neuropathologie, G.H. Pitié-Salpêtrière, 75013 Paris, France. stephane.haik@upmc.fr. |
| Abstrakt: |
Prions are atypical infectious agents lacking genetic material. Yet, various strains have been isolated from animals and humans using experimental models. They are distinguished by the resulting pattern of disease, including the localization of PrPsc deposits and the spongiform changes they induce in the brain of affected individuals. In this paper, we discuss the emerging use of cellular and acellular models to decipher the mechanisms involved in the strain-specific targeting of distinct brain regions. Recent studies suggest that neuronal cultures, protein misfolding cyclic amplification, and combination of both approaches may be useful to explore this under-investigated but central domain of the prion field. |
