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Articles, 3 records found
Articles 3 records found  
1.
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, Mariví (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 Díez, 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)  
2.
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  
3.
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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4 Charco, Jorge M.
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