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A Protein Misfolding Shaking Amplification-based method for the spontaneous generation of hundreds of bona fide prions.
Eraña, Hasier; Sampedro-Torres-Quevedo, Cristina; Charco, Jorge M; Díaz-Domínguez, Carlos M; Peccati, Francesca; San-Juan-Ansoleaga, Maitena; Vidal, Enric; Gonçalves-Anjo, Nuno; Pérez-Castro, Miguel A; González-Miranda, Ezequiel; Piñeiro, Patricia; Fernández-Veiga, Leire; Galarza-Ahumada, Josu; Fernández-Muñoz, Eva; Perez de Nanclares, Guiomar; Telling, Glenn; Geijo, Mariví; Jiménez-Osés, Gonzalo; Castilla, Joaquín.
Afiliación
  • Eraña H; Center for Cooperative Research in Biosciences (CIC BioGUNE), Basque Research and Technology Alliance (BRTA), Derio, Spain.
  • Sampedro-Torres-Quevedo C; Centro de Investigación Biomédica en Red de Enfermedades infecciosas (CIBERINFEC), Carlos III National Health Institute, Madrid, Spain.
  • Charco JM; ATLAS Molecular Pharma S. L, Derio, Spain.
  • Díaz-Domínguez CM; Center for Cooperative Research in Biosciences (CIC BioGUNE), Basque Research and Technology Alliance (BRTA), Derio, Spain.
  • Peccati F; Center for Cooperative Research in Biosciences (CIC BioGUNE), Basque Research and Technology Alliance (BRTA), Derio, Spain.
  • San-Juan-Ansoleaga M; Centro de Investigación Biomédica en Red de Enfermedades infecciosas (CIBERINFEC), Carlos III National Health Institute, Madrid, Spain.
  • Vidal E; ATLAS Molecular Pharma S. L, Derio, Spain.
  • Gonçalves-Anjo N; Center for Cooperative Research in Biosciences (CIC BioGUNE), Basque Research and Technology Alliance (BRTA), Derio, Spain.
  • Pérez-Castro MA; Centro de Investigación Biomédica en Red de Enfermedades infecciosas (CIBERINFEC), Carlos III National Health Institute, Madrid, Spain.
  • González-Miranda E; Center for Cooperative Research in Biosciences (CIC BioGUNE), Basque Research and Technology Alliance (BRTA), Derio, Spain.
  • Piñeiro P; Center for Cooperative Research in Biosciences (CIC BioGUNE), Basque Research and Technology Alliance (BRTA), Derio, Spain.
  • Fernández-Veiga L; IRTA. Programa de Sanitat Animal. Centre de Recerca en Sanitat Animal (CReSA). Campus de la Universitat Autònoma de Barcelona (UAB), Bellaterra, Catalonia, Spain.
  • Galarza-Ahumada J; Unitat mixta d'Investigació IRTA-UAB en Sanitat Animal. Centre de Recerca en Sanitat Animal (CReSA). Campus de la Universitat Autònoma de Barcelona (UAB), Bellaterra, Catalonia, Spain.
  • Fernández-Muñoz E; Center for Cooperative Research in Biosciences (CIC BioGUNE), Basque Research and Technology Alliance (BRTA), Derio, Spain.
  • Perez de Nanclares G; Center for Cooperative Research in Biosciences (CIC BioGUNE), Basque Research and Technology Alliance (BRTA), Derio, Spain.
  • Telling G; Center for Cooperative Research in Biosciences (CIC BioGUNE), Basque Research and Technology Alliance (BRTA), Derio, Spain.
  • Geijo M; Center for Cooperative Research in Biosciences (CIC BioGUNE), Basque Research and Technology Alliance (BRTA), Derio, Spain.
  • Jiménez-Osés G; Center for Cooperative Research in Biosciences (CIC BioGUNE), Basque Research and Technology Alliance (BRTA), Derio, Spain.
  • Castilla J; Center for Cooperative Research in Biosciences (CIC BioGUNE), Basque Research and Technology Alliance (BRTA), Derio, Spain.
Nat Commun ; 15(1): 2112, 2024 Mar 08.
Article en En | MEDLINE | ID: mdl-38459071
ABSTRACT
Prion diseases are a group of rapidly progressing neurodegenerative disorders caused by the misfolding of the endogenous prion protein (PrPC) into a pathogenic form (PrPSc). 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.
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Texto completo: 1 Colección: 01-internacional Base de datos: MEDLINE Asunto principal: Priones / Enfermedades por Prión Límite: Animals Idioma: En Revista: Nat Commun Asunto de la revista: BIOLOGIA / CIENCIA Año: 2024 Tipo del documento: Article País de afiliación: España Pais de publicación: Reino Unido
Texto completo
Añadir a Mi BVS
Imprimir
XML
PubMed Links
Buscar en Google

Texto completo: 1 Colección: 01-internacional Base de datos: MEDLINE Asunto principal: Priones / Enfermedades por Prión Límite: Animals Idioma: En Revista: Nat Commun Asunto de la revista: BIOLOGIA / CIENCIA Año: 2024 Tipo del documento: Article País de afiliación: España Pais de publicación: Reino Unido