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An in vivo Caenorhabditis elegans model for therapeutic research in human prion diseases
Brain  (IF13.501),  Pub Date : 2021-10-21, DOI: 10.1093/brain/awab152
Nicolas Bizat, Valeria Parrales, Sofian Laoues, Sébastien Normant, Etienne Levavasseur, Julian Roussel, Nicolas Privat, Alexianne Gougerot, Philippe Ravassard, Patrice Beaudry, Jean-Philippe Brandel, Jean-Louis Laplanche, Stéphane Haïk

Human prion diseases are fatal neurodegenerative disorders that include sporadic, infectious and genetic forms. Inherited Creutzfeldt-Jakob disease due to the E200K mutation of the prion protein-coding gene is the most common form of genetic prion disease. The phenotype resembles that of sporadic Creutzfeldt-Jakob disease at both the clinical and pathological levels, with a median disease duration of 4 months. To date, there is no available treatment for delaying the occurrence or slowing the progression of human prion diseases. Existing in vivo models do not allow high-throughput approaches that may facilitate the discovery of compounds targeting pathological assemblies of human prion protein or their effects on neuronal survival.