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| Pàgina inicial > Articles > Articles publicats > Understanding the key features of the spontaneous formation of bona fide prions through a novel methodology that enables their swift and consistent generation |
(Centro de Investigación Biomédica en Red de Enfermedades Infecciosas) (Centro de Investigación Biomédica en Red de Enfermedades Infecciosas) (Centro de Investigación Biomédica en Red de Enfermedades Infecciosas) (Unitat mixta d'investigació IRTA-UAB en Sanitat Animal. Centre de Recerca en Sanitat Animal) (Basque Research and Technology Alliance) (Basque Research and Technology Alliance) (Basque Research and Technology Alliance) (Basque Research and Technology Alliance) (Basque Research and Technology Alliance) (Basque Research and Technology Alliance) (Basque Research and Technology Alliance) (IKERBASQUE. Basque Foundation for Science)
| Data: | 2023 |
| Resum: | Among transmissible spongiform encephalopathies or prion diseases affecting humans, sporadic forms such as sporadic Creutzfeldt-Jakob disease are the vast majority. Unlike genetic or acquired forms of the disease, these idiopathic forms occur seemingly due to a random event of spontaneous misfolding of the cellular PrP (PrPC) into the pathogenic isoform (PrPSc). Currently, the molecular mechanisms that trigger and drive this event, which occurs in approximately one individual per million each year, remain completely unknown. Modelling this phenomenon in experimental settings is highly challenging due to its sporadic and rare occurrence. Previous attempts to model spontaneous prion misfolding in vitro have not been fully successful, as the spontaneous formation of prions is infrequent and stochastic, hindering the systematic study of the phenomenon. In this study, we present the first method that consistently induces spontaneous misfolding of recombinant PrP into bona fide prions within hours, providing unprecedented possibilities to investigate the mechanisms underlying sporadic prionopathies. By fine-tuning the Protein Misfolding Shaking Amplification method, which was initially developed to propagate recombinant prions, we have created a methodology that consistently produces spontaneously misfolded recombinant prions in 100% of the cases. Furthermore, this method gives rise to distinct strains and reveals the critical influence of charged surfaces in this process. |
| Ajuts: | Agencia Estatal de Investigación PID2021-122201OB-C21 Agencia Estatal de Investigación PID2021-1222010B-C22 Agencia Estatal de Investigación PID2020-117465GB-I00 Instituto de Salud Carlos III AC21_2/00024 |
| Drets: | Aquest document està subjecte a una llicència d'ús Creative Commons. Es permet la reproducció total o parcial, la distribució, la comunicació pública de l'obra i la creació d'obres derivades, fins i tot amb finalitats comercials, sempre i quan es reconegui l'autoria de l'obra original.  |
| Llengua: | Anglès |
| Document: | Article ; recerca ; Versió publicada |
| Matèria: | Prion ; Transmissible spongiform encephalopathies ; Spontaneous misfolding ; Strain |
| Publicat a: | Acta neuropathologica communications, Vol. 11 (september 2023) , ISSN 2051-5960 |
