Results overview: Found 8 records in 0.03 seconds.
Articles, 8 records found
Articles 8 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.
8 p, 941.3 KB Classical BSE prions emerge from asymptomatic pigs challenged with atypical/Nor98 scrapie / Marín, Belén (Centro de Encefalopatías y Enfermedades Transmisibles Emergentes) ; Otero, Alicia (Universidad de Zaragoza. Centro de Encefalopatías y Enfermedades Transmisibles Emergentes) ; Lugan, Séverine (École Nationale Vétérinaire de Toulouse) ; Espinosa, Juan Carlos (Centro de Investigación en Sanidad Animal. CISA (Madrid, Espanya)) ; Marín-Moreno, Alba (Centro de Investigación en Sanidad Animal. CISA (Madrid, Espanya)) ; Vidal Barba, Enric (Institut de Recerca i Tecnologia Agroalimentàries. Centre de Recerca en Sanitat Animal) ; Hedman, Carlos (Centro de Encefalopatías y Enfermedades Transmisibles Emergentes) ; Romero, Antonio (Universidad de Zaragoza. Servicio de Cirugía y Medicina Equina) ; Pumarola i Batlle, Martí (Universitat Autònoma de Barcelona. Departament de Medicina i Cirurgia Animals) ; Badiola, Juan José (Universidad de Zaragoza. Centro de Encefalopatías y Enfermedades Transmisibles Emergentes) ; Torres, Juan María (Centro de Investigación en Sanidad Animal. CISA (Madrid, Espanya)) ; Andreoletti, Olivier (École Nationale Vétérinaire de Toulouse) ; Bolea, Rosa (Universidad de Zaragoza. Centro de Encefalopatías y Enfermedades Transmisibles Emergentes)
Pigs are susceptible to infection with the classical bovine spongiform encephalopathy (C-BSE) agent following experimental inoculation, and PrPSc accumulation was detected in porcine tissues after the inoculation of certain scrapie and chronic wasting disease isolates. [...]
2021 - 10.1038/s41598-021-96818-2
Scientific reports, Vol. 11 Núm. 1 (2021)  
4.
10 p, 1.5 MB The emergence of classical BSE from atypical/Nor98 scrapie / Huor, Alvina (École Nationale Vétérinaire de Toulouse) ; Espinosa, Juan Carlos (Centro de Investigación en Sanidad Animal-Instituto Nacional de Investigación y Tecnología Agraria y Alimentaria) ; Vidal Barba, Enric (Institut de Recerca i Tecnologia Agroalimentàries. Centre de Recerca en Sanitat Animal) ; Cassard, Hervé (École Nationale Vétérinaire de Toulouse) ; Douet, Jean-Yves (École Nationale Vétérinaire de Toulouse) ; Lugan, Séverine (École Nationale Vétérinaire de Toulouse) ; Aron, Naima (École Nationale Vétérinaire de Toulouse) ; Marín-Moreno, Alba (Centro de Investigación en Sanidad Animal-Instituto Nacional de Investigación y Tecnología Agraria y Alimentaria) ; Lorenzo, Patricia (Centro de Investigación en Sanidad Animal-Instituto Nacional de Investigación y Tecnología Agraria y Alimentaria) ; Aguilar-Calvo, Patricia (Centro de Investigación en Sanidad Animal-Instituto Nacional de Investigación y Tecnología Agraria y Alimentaria) ; Badiola, Juan José (Centro de Encefalopatías y Enfermedades Transmisibles Emergentes) ; Bolea, Rosa (Centro de Encefalopatías y Enfermedades Transmisibles Emergentes) ; Pumarola i Batlle, Martí (Universitat Autònoma de Barcelona. Unitat de Patologia Murina i Comparada) ; Benestad, Sylvie L. (Norwegian Veterinary Institute) ; Orge, Leonore (National Institute for Agrarian and Veterinary Research) ; Thackray, Alana M. (University of Cambridge) ; Bujdoso, Raymond (University of Cambridge) ; Torres, Juan-Maria (Centro de Investigación en Sanidad Animal-Instituto Nacional de Investigación y Tecnología Agraria y Alimentaria) ; Andreoletti, Olivier (University of Cambridge)
Atypical/Nor98 scrapie (AS) is a prion disease of small ruminants. Currently there are no efficient measures to control this form of prion disease, and, importantly, the zoonotic potential and the risk that AS might represent for other farmed animal species remains largely unknown. [...]
2019 - 10.1073/pnas.1915737116
The Proceedings of the National Academy of Sciences (PNAS), Vol. 116 Núm. 52 (2019)  
5.
15 p, 4.2 MB Mixtures of prion substrains in natural scrapie cases revealed by ovinised murine models / Barrio, Tomás (Universidad de Zaragoza. Centro de Encefalopatías y Enfermedades Transmisibles Emergentes) ; Filali, Hicham (Universidad de Zaragoza. Centro de Encefalopatías y Enfermedades Transmisibles Emergentes) ; Otero García, Alicia (Universidad de Zaragoza. Centro de Encefalopatías y Enfermedades Transmisibles Emergentes) ; Sheleby-Elías, Jessica (Universidad de Zaragoza. Centro de Encefalopatías y Enfermedades Transmisibles Emergentes) ; Marín, Belén (Universidad de Zaragoza. Centro de Encefalopatías y Enfermedades Transmisibles Emergentes) ; Vidal Barba, Enric (Institut de Recerca i Tecnologia Agroalimentàries. Centre de Recerca en Sanitat Animal) ; Béringue, Vincent (Université Paris-Saclay. Virologie Immunologie Moléculaires) ; Torres, Juan María (Centro de Investigación en Sanidad Animal. CISA (Madrid, Espanya)) ; Groschup, Martin (Friedrich-Loeffler-Institute. Institute of Novel and Emerging Infectious Diseases) ; Andreoletti, Olivier (École Nationale Vétérinaire de Toulouse) ; Badiola, Juan José (Universidad de Zaragoza. Centro de Encefalopatías y Enfermedades Transmisibles Emergentes) ; Bolea, Rosa (Universidad de Zaragoza. Centro de Encefalopatías y Enfermedades Transmisibles Emergentes)
Phenotypic variability in prion diseases, such as scrapie, is associated to the existence of prion strains, which are different pathogenic prion protein (PrP Sc) conformations with distinct pathobiological properties. [...]
2020 - 10.1038/s41598-020-61977-1
Scientific reports, Vol. 10 (march 2020)  
6.
19 p, 6.1 MB TREM2 expression in the brain and biological fluids in prion diseases / Diaz-Lucena, Daniela (Institut d'Investigació Biomèdica de Bellvitge) ; Kruse, Niels (University Medical Center Göttingen. Institute of Neuropathology) ; Thüne, Katrin (University Medical Center Göttingen. Department of Neurology) ; Schmitz, Matthias (German Center for Neurodegenerative Diseases) ; Villar-Piqué, Anna (Institut d'Investigació Biomèdica de Bellvitge) ; da Cunha, Jose Eriton Gomes (Universidade Federal de Pernambuco. Keizo Asami Laboratory) ; Hermann, Peter (University Medical Center Göttingen. Department of Neurology) ; López-Pérez, Óscar (Institut d'Investigació Biomèdica de Bellvitge) ; Andrés-Benito, P (Institut d'Investigació Biomèdica de Bellvitge) ; Ladogana, Anna (Istituto Superiore Di Sanità. Department of Neurosciences) ; Calero, Miguel (Centro de Investigación Biomédica en Red sobre Enfermedades Neurodegenerativas) ; Vidal Barba, Enric (Institut de Recerca i Tecnologia Agroalimentàries) ; Riggert, Joachim (University Medical School. Department of Transfusion Medicine) ; Pineau, Hailey (University of Alberta. Department of Medicine-Division of Neurology) ; Sim, Valerie (University of Alberta. Department of Medicine-Division of Neurology) ; Zetterberg, Henrik (UCL Institute of Neurology (Regne Unit)) ; Blennow, Kaj (Sahlgrenska University Hospital. Clinical Neurochemistry Laboratory) ; Del Rio, Jose Antonio (Universitat de Barcelona. Institut de Neurociències) ; Marín-Moreno, Alba (Centro de Investigación en Sanidad Animal. CISA-INIA) ; Espinosa, Juan Carlos (Centro de Investigación en Sanidad Animal. CISA-INIA) ; Torres, Juan María (Centro de Investigación en Sanidad Animal. CISA-INIA) ; Sanchez-Valle, Raquel (Institut d'Investigacions Biomèdiques August Pi i Sunyer) ; Mollenhauer, Brit (University Medical Centre Göttingen. Department of Neurology) ; Ferrer, Isidro (Universitat de Barcelona. Departament de Patologia i Terapèutica Experimental) ; Zerr, Inga (German Center for Neurodegenerative Diseases) ; Llorens, Franc (University Medical Center Göttingen. Department of Neurology)
Triggering receptor expressed on myeloid cells 2 (TREM2) is an innate immune cell surface receptor that regulates microglial function and is involved in the pathophysiology of several neurodegenerative diseases. [...]
2021 - 10.1007/s00401-021-02296-1
Acta Neuropathologica, Vol. 141 (april 2021) , p. 841-859  
7.
9 p, 2.0 MB Bovine Spongiform Encephalopathy Induces Misfolding of Alleged Prion-Resistant Species Cellular Prion Protein without Altering Its Pathobiological Features / Vidal Barba, Enric (Institut de Recerca i Tecnologia Agroalimentàries. Centre de Recerca en Sanitat Animal) ; Fernández-Borges, Natalia ; Pintado, Belén ; Ordóñez, Montserrat (Institut de Recerca i Tecnologia Agroalimentàries. Centre de Recerca en Sanitat Animal) ; Márquez, Mercedes ; Fondevila, Dolors (Universitat Autònoma de Barcelona. Departament de Medicina i Cirurgia Animals) ; Torres, Juan María ; Pumarola i Batlle, Martí (Universitat Autònoma de Barcelona. Departament de Medicina i Cirurgia Animals) ; Castilla, Joaquín
Bovine spongiform encephalopathy (BSE) prions were responsible for an unforeseen epizootic in cattle which had a vast social, economic, and public health impact. This was primarily because BSE prions were found to be transmissible to humans. [...]
2013 - 10.1523/JNEUROSCI.0244-13.2013
The Journal of Neuroscience, Vol. 33 Núm. 18 (May 2013) , p. 7778-7786  
8.
14 p, 2.7 MB Central nervous system gene expression changes in a transgenic mouse model for bovine spongiform encephalopathy / Tortosa Méndez, Raül (Universitat Autònoma de Barcelona. Departament de Medicina i Cirurgia Animals) ; Castells, Xavier (Universitat Autònoma de Barcelona. Institut de Biotecnologia i de Biomedicina "Vicent Villar Palasí") ; Vidal Barba, Enric (Institut de Recerca i Tecnologia Agroalimentàries. Centre de Recerca en Sanitat Animal) ; Costa Riu, Carme (Universitat Autònoma de Barcelona. Departament de Medicina i Cirurgia Animals) ; Ruiz de Villa, María del Carmen (Universitat de Barcelona. Facultat de Biologia) ; Sánchez, Alex (Universitat de Barcelona. Facultat de Biologia) ; Barceló Vernet, Anna (Universitat Autònoma de Barcelona. Departament de Bioquímica i de Biologia Molecular) ; Torres, Juan María (Instituto Nacional de Investigaciones Agronómicas (Espanya)) ; Pumarola i Batlle, Martí (Universitat Autònoma de Barcelona. Departament de Medicina i Cirurgia Animals) ; Ariño Carmona, Joaquín (Universitat Autònoma de Barcelona. Departament de Bioquímica i de Biologia Molecular)
Gene expression analysis has proven to be a very useful tool to gain knowledge of the factors involved in the pathogenesis of diseases, particularly in the initial or preclinical stages. With the aim of finding new data on the events occurring in the Central Nervous System in animals affected with Bovine Spongiform Encephalopathy, a comprehensive genome wide gene expression study was conducted at different time points of the disease on mice genetically modified to model the bovine species brain in terms of cellular prion protein. [...]
2011 - 10.1186/1297-9716-42-109
Veterinary research, Vol. 42, N. 109 (October 2011) , p. 1-14  

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