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Pàgina inicial > Articles > Articles publicats > A Protein Misfolding Shaking Amplification-based method for the spontaneous generation of hundreds of bona fide prions |
Data: | 2024 |
Resum: | Prion diseases are a group of rapidly progressing neurodegenerative disorders caused by the misfolding of the endogenous prion protein (PrP C) into a pathogenic form (PrP Sc). This process, despite being the central event underlying these disorders, remains largely unknown at a molecular level, precluding the prediction of new potential outbreaks or interspecies transmission incidents. In this work, we present a method to generate bona fide recombinant prions de novo, allowing a comprehensive analysis of protein misfolding across a wide range of prion proteins from mammalian species. We study more than 380 different prion proteins from mammals and classify them according to their spontaneous misfolding propensity and their conformational variability. This study aims to address fundamental questions in the prion research field such as defining infectivity determinants, interspecies transmission barriers or the structural influence of specific amino acids and provide invaluable information for future diagnosis and therapy applications. To study neurodegenerative prion diseases, a method (PMSA) for generating prions spontaneously is presented. Applied to 380+ different prion proteins, their tendency to become pathogenic was ranked, illuminating their formation process. |
Ajuts: | Ministerio de Economía y Competitividad PID2021-122201OB-C21 Ministerio de Economía y Competitividad PID2021-1222010B-C22 Ministerio de Economía y Competitividad PID2021-125946OB-I00 Agencia Estatal de Investigación IJC2020-045506-I |
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: | Neurodegeneration ; Diseases of the nervous system ; Encephalopathy ; Prions ; Protein aggregation |
Publicat a: | Nature communications, Vol. 15 (march 2024) , ISSN 2041-1723 |
14 p, 2.1 MB |