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Articles, 6 registres trobats
Articles 6 registres trobats  
1.
14 p, 2.1 MB A Protein Misfolding Shaking Amplification-based method for the spontaneous generation of hundreds of bona fide prions / Eraña, Hasier (ATLAS Molecular Pharma S. L) ; Sampedro-Torres-Quevedo, Cristina (Basque Research and Technology Alliance) ; Charco, Jorge M. (ATLAS Molecular Pharma S. L) ; Díaz-Domínguez, Carlos M. (Instituto de Salut Carlos III. Centro de Investigación Biomédica en Red de Enfermedades infecciosas) ; Peccati, Francesca (Basque Research and Technology Alliance) ; San-Juan-Ansoleaga, Maitena (Basque Research and Technology Alliance) ; Vidal Barba, Enric (Unitat mixta d'investigació IRTA-UAB en Sanitat Animal. Centre de Recerca en Sanitat Animal) ; Gonçalves-Anjo, Nuno (Basque Research and Technology Alliance) ; Pérez-Castro, Miguel A. (Basque Research and Technology Alliance) ; González-Miranda, Ezequiel (Basque Research and Technology Alliance) ; Piñeiro, Patricia (Basque Research and Technology Alliance) ; Fernández-Veiga, Leire (Basque Research and Technology Alliance) ; Galarza-Ahumada, Josu (Basque Research and Technology Alliance) ; Fernández-Muñoz, Eva (Basque Research and Technology Alliance) ; Pérez de Nanclares, Guiomar (Hospital Universitario de Araba Molecular (Epi)Genetics Laboratory) ; Telling, Glenn (Colorado State University. Prion Research Center) ; Geijo, Maria V (NEIKER-Basque Institute for Agricultural Research and Development) ; Jiménez-Osés, Gonzalo (Basque Foundation for Science) ; Castilla, Joaquín (Basque Foundation for Science)
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. [...]
2024 - 10.1038/s41467-024-46360-2
Nature communications, Vol. 15 (march 2024)  
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2.
22 p, 3.0 MB Bona fide atypical scrapie faithfully reproduced for the first time in a rodent model / Vidal Barba, Enric (Unitat mixta d'investigació IRTA-UAB en Sanitat Animal. Centre de Recerca en Sanitat Animal) ; Sánchez-Martín, Manuel A. (Universidad de Salamanca. Departamento de Medicina) ; Eraña, Hasier (Instituto de Salud Carlos III. Centro de Investigación Biomédica en Red de Enfermedades Infecciosas (CIBERINFEC)) ; Lázaro, Sonia Pérez (Universidad de Zaragoza. Centro de Encefalopatías y Enfermedades Transmisibles Emergentes) ; Pérez-Castro, Miguel A. (Basque Centre for Climate Change (Bilbao)) ; Otero García, Alicia (Universidad de Zaragoza. Centro de Encefalopatías y Enfermedades Transmisibles Emergentes) ; Charco, Jorge M. (Centro de Investigación Biomédica en Red de Enfermedades Infecciosas) ; Marín, Belén (Universidad de Zaragoza. Centro de Encefalopatías y Enfermedades Transmisibles Emergentes) ; López-Moreno, Rafael (Basque Research and Technology Alliance (BRTA). Centro de Investigación Cooperativa en Biociencias) ; Díaz-Domínguez, Carlos M. (Basque Centre for Climate Change (Bilbao)) ; Geijo, Maria V (NEIKER-Basque Institute for Agricultural Research and Development) ; Ordóñez, Montserrat (Institut de Recerca i Tecnologia Agroalimentàries. Centre de Recerca en Sanitat Animal) ; Cantero, Guillermo (Institut de Recerca i Tecnologia Agroalimentàries. Centre de Recerca en Sanitat Animal) ; di Bari, Michele (Istituto Superiore Di Sanità. Department of Food Safety, Nutrition and Veterinary Public Health) ; Lorenzo, Nuria L. (Universidade de Santiago de Compostela) ; Pirisinu, Laura (Istituto Superiore Di Sanità. Department of Food Safety, Nutrition and Veterinary Public Health) ; d'Agostino, Claudia (Istituto Superiore Di Sanità. Department of Food Safety, Nutrition and Veterinary Public Health) ; Torres, Juan María (Instituto Nacional de Investigación y Tecnología Agraria y Alimentaria. Centro de Investigación en Sanidad Animal) ; Béringue, Vincent (Université Paris-Saclay. Institut National de La Recherche Agronomique) ; Telling, Glenn (Colorado State University. Prion Research Center and the Department of Microbiology, Immunology, and Pathology) ; Badiola, Juan José (Universidad de Zaragoza. Centro de Encefalopatías y Enfermedades Transmisibles Emergentes) ; Pumarola i Batlle, Martí (Universitat Autònoma de Barcelona. Departament de Medicina i Cirurgia Animals) ; Bolea, Rosa (Universidad de Zaragoza. Centro de Encefalopatías y Enfermedades Transmisibles Emergentes) ; Nonno, Romolo (Istituto Superiore Di Sanità. Department of Food Safety, Nutrition and Veterinary Public Health) ; Requena, Jesús R. (Universidade de Santiago de Compostela) ; Castilla, Joaquín (IKERBASQUE. Basque Foundation for Science)
Atypical Scrapie, which is not linked to epidemics, is assumed to be an idiopathic spontaneous prion disease in small ruminants. Therefore, its occurrence is unlikely to be controlled through selective breeding or other strategies as it is done for classical scrapie outbreaks. [...]
2022 - 10.1186/s40478-022-01477-7
Acta neuropathologica communications, Vol. 10 (december 2022)  
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3.
13 p, 3.2 MB Homozygous R136S mutation in PRNP gene causes inherited early onset prion disease / Ximelis, Teresa (Institut d'Investigacions Biomèdiques August Pi i Sunyer) ; Marín-Moreno, Alba (Centro de Investigación en Sanidad Animal (CISA-INIA-CSIC)) ; Espinosa, J. C. (Centro de Investigación en Sanidad Animal (CISA-INIA-CSIC)) ; Eraña, Hasier (Center for Cooperative Research in Biosciences (CIC bioGUNE). Basque Research and Technology Alliance (BRTA)) ; Charco, Jorge M. (Center for Cooperative Research in Biosciences (CIC bioGUNE). Basque Research and Technology Alliance (BRTA)) ; Hernández, Isabel (Ace Alzheimer Center Barcelona) ; Riveira, Carmen (Neurology Service. Hospital de León) ; Alcolea, Daniel (Institut d'Investigació Biomèdica Sant Pau) ; González-Roca, Eva (Hospital Clínic i Provincial de Barcelona) ; Aldecoa, Iban (Hospital Clínic i Provincial de Barcelona) ; Molina-Porcel, Laura (Institut d'Investigacions Biomèdiques August Pi i Sunyer) ; Parchi, Piero (IRCCS. Istituto delle Scienze Neurologiche di Bologna) ; Rossi, Marcello (IRCCS. Istituto delle Scienze Neurologiche di Bologna) ; Castilla, Joaquín (IKERBasque Basque Foundation for Science) ; Ruiz-García, Raquel (Institut d'Investigacions Biomèdiques August Pi i Sunyer) ; Gelpi, Ellen (Medical University of Vienna) ; Torres, Juan María (Centro de Investigación en Sanidad Animal (CISA-INIA-CSIC)) ; Sanchez-Valle, Raquel (Institut d'Investigacions Biomèdiques August Pi i Sunyer) ; Universitat Autònoma de Barcelona
Background: More than 40 pathogenic heterozygous PRNP mutations causing inherited prion diseases have been identified to date. Recessive inherited prion disease has not been described to date. Methods: We describe the clinical and neuropathological data of inherited early-onset prion disease caused by the rare PRNP homozygous mutation R136S. [...]
2021 - 10.1186/s13195-021-00912-6
Alzheimer's research & therapy, Vol. 13 Núm. 1 (december 2021) , p. 176  
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4.
27 p, 13.9 MB Unraveling the key to the resistance of canids to prion diseases / Fernández-Borges, Natalia (CIC bioGUNE. Parque tecnológico de Bizkaia) ; Parra, Beatriz (Laboratorio Central de Veterinaria) ; Vidal Barba, Enric (Institut de Recerca i Tecnologia Agroalimentàries. Centre de Recerca en Sanitat Animal) ; Eraña, Hasier (CIC bioGUNE. Parque tecnológico de Bizkaia) ; Sánchez-Martín, Manuel A. (Instituto de Investigación Biomédica de Salamanca) ; de Castro, Jorge (Department of Infectology) ; Elezgarai, Saioa R. (CIC bioGUNE. Parque tecnológico de Bizkaia) ; Pumarola i Batlle, Martí (Universitat Autònoma de Barcelona. Departament de Medicina i Cirurgia Animals) ; Mayoral, Tomás (Laboratorio Central de Veterinaria) ; Castilla, Joaquín (Basque Foundation for Science)
One of the characteristics of prions is their ability to infect some species but not others and prion resistant species have been of special interest because of their potential in deciphering the determinants for susceptibility. [...]
2017 - 10.1371/journal.ppat.1006716
PLOS pathogens, Vol. 13 (november 2017)  
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5.
33 p, 2.7 MB Development of a new largely scalable in vitro prion propagation method for the production of infectious recombinant prions for high resolution structural studies / Eraña, Hasier (ATLAS Molecular Pharma S. L.) ; Charco, Jorge M. (CIC bioGUNE) ; Di Bari, Michele Angelo (CIC bioGUNE) ; Díaz-Domínguez, Carlos M. (Istituto Superiore di Sanità. Department of Veterinary Public Health and Food Safety.) ; López-Moreno, R. (CIC bioGUNE) ; Vidal Barba, Enric (Institut de Recerca i Tecnologia Agroalimentàries. Centre de Recerca en Sanitat Animal) ; González-Miranda, E. (CIC bioGUNE) ; Pérez-Castro, Miguel A. (CIC bioGUNE) ; García-Martínez, Sandra (ATLAS Molecular Pharma S. L.) ; Bravo Lopez, Susana Belén (Proteomics Lab. IDIS) ; Fernández-Borges, Natalia (CIC bioGUNE) ; Geijo, Maria V. (NEIKER-Instituto Vasco de Investigación y Desarrollo Agrario) ; D'Agostino, C. (Department of Veterinary Public Health and Food Safety. Istituto Superiore di Sanità) ; Garrido, Joseba M (NEIKER-Instituto Vasco de Investigación y Desarrollo Agrario) ; Bian, J. (Colorado State University. Prion Research Center (PRC).) ; König, A. (Heinrich-Heine-Universität Düsseldorf. Physikalische Biologie.) ; Uluca-Yazgi, B. (Heinrich-Heine-Universität Düsseldorf. Physikalische Biologie.) ; Sabate, R. (Universitat de Barcelona. Institut de Nanociència i Nanotecnologia) ; Khaychuk, V. (Colorado State University. Prion Research Center (PRC).) ; Vanni, Ilaria (Istituto Superiore di Sanità. Department of Veterinary Public Health and Food Safety.) ; Telling, G. C. (Colorado State University. Prion Research Center (PRC).) ; Heise, Henrike (Heinrich-Heine-Universität Düsseldorf. Physikalische Biologie.) ; Nonno, Romolo (Istituto Superiore di Sanità. Department of Veterinary Public Health and Food Safety.) ; Requena, Jesús R (Universidade de Santiago de Compostela) ; Castilla, Joaquín (IKERBasque. Basque Foundation for Science)
The resolution of the three-dimensional structure of infectious prions at the atomic level is pivotal to understand the pathobiology of Transmissible Spongiform Encephalopathies (TSE), but has been long hindered due to certain particularities of these proteinaceous pathogens. [...]
2019 - 10.1371/journal.ppat.1008117
PLOS pathogens, Vol. 15 Núm. 10 (2019) , p. e1008117  
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6.
26 p, 6.8 MB Transgenic Mouse Bioassay : Evidence That Rabbits Are Susceptible to a Variety of Prion Isolates / Vidal Barba, Enric (Institut de Recerca i Tecnologia Agroalimentàries. Centre de Recerca en Sanitat Animal) ; Fernández-Borges, Natalia ; Pintado, Belén ; Eraña, Hasier ; Ordóñez, Montserrat ; Márquez, Mercedes ; Chianini, Francesca ; Fondevila, Dolors (Universitat Autònoma de Barcelona. Departament de Medicina i Cirurgia Animals) ; Sánchez-Martín, Manuel A. ; Andreoletti, Olivier ; Dagleish, Mark P. ; Pumarola i Batlle, Martí (Universitat Autònoma de Barcelona. Departament de Medicina i Cirurgia Animals) ; Castilla, Joaquín
Interspecies transmission of prions is a well-established phenomenon, both experimentally and under field conditions. Upon passage through new hosts, prion strains have proven their capacity to change their properties and this is a source of strain diversity which needs to be considered when assessing the potential risks associated with consumption of prion contaminated protein sources. [...]
2015 - 10.1371/journal.ppat.1004977
PLOS pathogens, Núm. 11 (August 2015) , p. 1-21  
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