Proteomic Analysis of Human iPSC-Derived Neural Stem Cells and Motor Neurons Identifies Proteasome Structural Alterations.
Autorzy:
Álvarez I; Departament de Biologia Cellular, Institut de Biotecnologia i Biomedicina, Universitat Autònoma de Barcelona, Fisiologia i Immunologia, 08193 Barcelona, Spain. Tirado-Herranz A; Departament de Biologia Cellular, Institut de Biotecnologia i Biomedicina, Universitat Autònoma de Barcelona, Fisiologia i Immunologia, 08193 Barcelona, Spain. Alvarez-Palomo B; Banc de Sang i Teixits, Edifici Dr. Frederic Duran i Jordà, Passeig Taulat, 116, 08005 Barcelona, Spain. Osete JR; Department of Medical Genetics, Oslo University Hospital, 0450 Oslo, Norway.; NORMENT, Institute of Clinical Medicine, University of Oslo, 0316 Oslo, Norway.; Division of Mental Health and Addiction, Oslo University Hospital, 4956 Oslo, Norway. Edel MJ; Department of Anatomy and Embryology, Faculty of Medicine, Universitat Autònoma de Barcelona, 08193 Barcelona, Spain.; Discipline of Medical Sciences and Genetics, School of Biomedical Sciences, University of Western Australia, Perth 6009, Australia.
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Źródło:
Cells [Cells] 2023 Dec 08; Vol. 12 (24). Date of Electronic Publication: 2023 Dec 08.
Understanding the key features of the spontaneous formation of bona fide prions through a novel methodology that enables their swift and consistent generation.
Autorzy:
Eraña H; Center for Cooperative Research in Biosciences (CIC bioGUNE), Basque Research and Technology Alliance (BRTA), Bizkaia Technology Park, 48160, Derio, Bizkaia, Spain.; ATLAS Molecular Pharma S. L. Bizkaia Technology Park, 48160, Derio, Spain.; Centro de Investigación Biomédica en Red de Enfermedades Infecciosas (CIBERINFEC), Carlos III National Health Institute, 28029, Madrid, Spain. Díaz-Domínguez CM; Center for Cooperative Research in Biosciences (CIC bioGUNE), Basque Research and Technology Alliance (BRTA), Bizkaia Technology Park, 48160, Derio, Bizkaia, Spain.; Centro de Investigación Biomédica en Red de Enfermedades Infecciosas (CIBERINFEC), Carlos III National Health Institute, 28029, Madrid, Spain. Charco JM; Center for Cooperative Research in Biosciences (CIC bioGUNE), Basque Research and Technology Alliance (BRTA), Bizkaia Technology Park, 48160, Derio, Bizkaia, Spain.; ATLAS Molecular Pharma S. L. Bizkaia Technology Park, 48160, Derio, Spain.; Centro de Investigación Biomédica en Red de Enfermedades Infecciosas (CIBERINFEC), Carlos III National Health Institute, 28029, Madrid, Spain. Vidal E; IRTA, Programa de Sanitat Animal, Centre de Recerca en Sanitat Animal (CReSA), Campus de la Universitat Autònoma de Barcelona (UAB), Bellaterra, Catalonia, Spain. González-Miranda E; Center for Cooperative Research in Biosciences (CIC bioGUNE), Basque Research and Technology Alliance (BRTA), Bizkaia Technology Park, 48160, Derio, Bizkaia, Spain. Pérez-Castro MA; Center for Cooperative Research in Biosciences (CIC bioGUNE), Basque Research and Technology Alliance (BRTA), Bizkaia Technology Park, 48160, Derio, Bizkaia, Spain. Piñeiro P; Center for Cooperative Research in Biosciences (CIC bioGUNE), Basque Research and Technology Alliance (BRTA), Bizkaia Technology Park, 48160, Derio, Bizkaia, Spain. López-Moreno R; Center for Cooperative Research in Biosciences (CIC bioGUNE), Basque Research and Technology Alliance (BRTA), Bizkaia Technology Park, 48160, Derio, Bizkaia, Spain. Sampedro-Torres-Quevedo C; Center for Cooperative Research in Biosciences (CIC bioGUNE), Basque Research and Technology Alliance (BRTA), Bizkaia Technology Park, 48160, Derio, Bizkaia, Spain. Fernández-Veiga L; Center for Cooperative Research in Biosciences (CIC bioGUNE), Basque Research and Technology Alliance (BRTA), Bizkaia Technology Park, 48160, Derio, Bizkaia, Spain. Tasis-Galarza J; Center for Cooperative Research in Biosciences (CIC bioGUNE), Basque Research and Technology Alliance (BRTA), Bizkaia Technology Park, 48160, Derio, Bizkaia, Spain. Lorenzo NL; CIMUS Biomedical Research Institute and Department of Medical Sciences, University of Santiago de Compostela-IDIS, 15782, Santiago de Compostela, Spain. Santini-Santiago A; Center for Cooperative Research in Biosciences (CIC bioGUNE), Basque Research and Technology Alliance (BRTA), Bizkaia Technology Park, 48160, Derio, Bizkaia, Spain. Lázaro M; Center for Cooperative Research in Biosciences (CIC bioGUNE), Basque Research and Technology Alliance (BRTA), Bizkaia Technology Park, 48160, Derio, Bizkaia, Spain. García-Martínez S; Center for Cooperative Research in Biosciences (CIC bioGUNE), Basque Research and Technology Alliance (BRTA), Bizkaia Technology Park, 48160, Derio, Bizkaia, Spain. Gonçalves-Anjo N; Center for Cooperative Research in Biosciences (CIC bioGUNE), Basque Research and Technology Alliance (BRTA), Bizkaia Technology Park, 48160, Derio, Bizkaia, Spain. San-Juan-Ansoleaga M; Center for Cooperative Research in Biosciences (CIC bioGUNE), Basque Research and Technology Alliance (BRTA), Bizkaia Technology Park, 48160, Derio, Bizkaia, Spain. Galarza-Ahumada J; Center for Cooperative Research in Biosciences (CIC bioGUNE), Basque Research and Technology Alliance (BRTA), Bizkaia Technology Park, 48160, Derio, Bizkaia, Spain. Fernández-Muñoz E; Center for Cooperative Research in Biosciences (CIC bioGUNE), Basque Research and Technology Alliance (BRTA), Bizkaia Technology Park, 48160, Derio, Bizkaia, Spain. Giler S; IRTA, Programa de Sanitat Animal, Centre de Recerca en Sanitat Animal (CReSA), Campus de la Universitat Autònoma de Barcelona (UAB), Bellaterra, Catalonia, Spain. Valle M; Center for Cooperative Research in Biosciences (CIC bioGUNE), Basque Research and Technology Alliance (BRTA), Bizkaia Technology Park, 48160, Derio, Bizkaia, Spain. Telling GC; Prion Research Center (PRC), Colorado State University, Fort Collins, CO, 80523, USA. Geijó M; Animal Health Department, NEIKER-Basque Institute for Agricultural Research and Development, Basque Research and Technology Alliance (BRTA), Bizkaia Technology Park, 48160, Derio, Spain. RequenaJR; CIMUS Biomedical Research Institute and Department of Medical Sciences, University of Santiago de Compostela-IDIS, 15782, Santiago de Compostela, Spain. Castilla J; Center for Cooperative Research in Biosciences (CIC bioGUNE), Basque Research and Technology Alliance (BRTA), Bizkaia Technology Park, 48160, Derio, Bizkaia, Spain. .; Centro de Investigación Biomédica en Red de Enfermedades Infecciosas (CIBERINFEC), Carlos III National Health Institute, 28029, Madrid, Spain. .; IKERBASQUE, Basque Foundation for Science, 48011, Bilbao, Spain. .
Bona fide atypical scrapie faithfully reproduced for the first time in a rodent model.
Autorzy:
Vidal E; 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.; IRTA Programa de Sanitat Animal, Centre de Recerca en Sanitat Animal (CReSA), Campus de la Universitat Autònoma de Barcelona (UAB), Bellaterra, Catalonia, Spain. Sánchez-Martín MA; Transgenic Facility. Department of Medicine, University of Salamanca, 37007, Salamanca, Spain. Eraña H; Centro de Investigación Cooperativa en Biociencias (CIC BioGUNE), Laboratorio de Investigación de Priones, Basque Research and Technology Alliance (BRTA), Derio, Bizkaia, Spain.; ATLAS Molecular Pharma S. L., Derio, Bizkaia, Spain.; Centro de Investigación Biomédica en Red de Enfermedades Infecciosas (CIBERINFEC), Instituto de Salud Carlos III, Madrid, Spain. Lázaro SP; Centro de Encefalopatías y Enfermedades Transmisibles Emergentes, Facultad de Veterinaria, Universidad de Zaragoza-IA2, Zaragoza, Spain. Pérez-Castro MA; Centro de Investigación Cooperativa en Biociencias (CIC BioGUNE), Laboratorio de Investigación de Priones, Basque Research and Technology Alliance (BRTA), Derio, Bizkaia, Spain. Otero A; Centro de Encefalopatías y Enfermedades Transmisibles Emergentes, Facultad de Veterinaria, Universidad de Zaragoza-IA2, Zaragoza, Spain. Charco JM; Centro de Investigación Cooperativa en Biociencias (CIC BioGUNE), Laboratorio de Investigación de Priones, Basque Research and Technology Alliance (BRTA), Derio, Bizkaia, Spain.; ATLAS Molecular Pharma S. L., Derio, Bizkaia, Spain.; Centro de Investigación Biomédica en Red de Enfermedades Infecciosas (CIBERINFEC), Instituto de Salud Carlos III, Madrid, Spain. Marín B; Centro de Encefalopatías y Enfermedades Transmisibles Emergentes, Facultad de Veterinaria, Universidad de Zaragoza-IA2, Zaragoza, Spain. López-Moreno R; Centro de Investigación Cooperativa en Biociencias (CIC BioGUNE), Laboratorio de Investigación de Priones, Basque Research and Technology Alliance (BRTA), Derio, Bizkaia, Spain. Díaz-Domínguez CM; Centro de Investigación Cooperativa en Biociencias (CIC BioGUNE), Laboratorio de Investigación de Priones, Basque Research and Technology Alliance (BRTA), Derio, Bizkaia, Spain. Geijo M; Animal Health Department, NEIKER-Basque Institute for Agricultural Research and Development, Basque Research and Technology Alliance (BRTA), Derio, Spain. Ordóñez M; 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.; IRTA Programa de Sanitat Animal, Centre de Recerca en Sanitat Animal (CReSA), Campus de la Universitat Autònoma de Barcelona (UAB), Bellaterra, Catalonia, Spain. Cantero G; 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.; IRTA Programa de Sanitat Animal, Centre de Recerca en Sanitat Animal (CReSA), Campus de la Universitat Autònoma de Barcelona (UAB), Bellaterra, Catalonia, Spain. di Bari M; Department of Food Safety, Nutrition and Veterinary Public Health, Istituto Superiore Di Sanità, 00161, Rome, Italy. Lorenzo NL; CIMUS Biomedical Research Institute, University of Santiago de Compostela-IDIS, Santiago, Spain. Pirisinu L; Department of Food Safety, Nutrition and Veterinary Public Health, Istituto Superiore Di Sanità, 00161, Rome, Italy. d'Agostino C; Department of Food Safety, Nutrition and Veterinary Public Health, Istituto Superiore Di Sanità, 00161, Rome, Italy. Torres JM; Centro de Investigación en Sanidad Animal (CISA), Centro Superior de Investigaciones Científicas (CSIC) Valdeolmos, Instituto Nacional de Investigación y Tecnología Agraria y Alimentaria (INIA), 28130, Madrid, Spain. Béringue V; Molecular Virology and Immunology, Institut National de La Recherche Agronomique (INRA), Université Paris-Saclay, Jouy-en-Josas, France. Telling G; Prion Research Center (PRC) and the Department of Microbiology, Immunology, and Pathology, Colorado State University, Fort Collins, CO, USA. Badiola JJ; Centro de Encefalopatías y Enfermedades Transmisibles Emergentes, Facultad de Veterinaria, Universidad de Zaragoza-IA2, Zaragoza, Spain. Pumarola M; Departament de Medicina i Cirurgia Animals, Facultat de Veterinària, Campus de UAB, Bellaterra, 08193, Barcelona, Catalonia, Spain. Bolea R; Centro de Encefalopatías y Enfermedades Transmisibles Emergentes, Facultad de Veterinaria, Universidad de Zaragoza-IA2, Zaragoza, Spain. Nonno R; Department of Food Safety, Nutrition and Veterinary Public Health, Istituto Superiore Di Sanità, 00161, Rome, Italy. RequenaJR; CIMUS Biomedical Research Institute, University of Santiago de Compostela-IDIS, Santiago, Spain. Castilla J; Centro de Investigación Cooperativa en Biociencias (CIC BioGUNE), Laboratorio de Investigación de Priones, Basque Research and Technology Alliance (BRTA), Derio, Bizkaia, Spain. .; Centro de Investigación Biomédica en Red de Enfermedades Infecciosas (CIBERINFEC), Instituto de Salud Carlos III, Madrid, Spain. .; IKERBASQUE, Basque Foundation for Science, Bilbao, Bizkaia, Spain. .
Celia's Encephalopathy ( BSCL2 -Gene-Related): Current Understanding.
Autorzy:
Sánchez-Iglesias S; UETeM-Molecular Pathology Group, Department of Psychiatry, Radiology, Public Health, Nursing and Medicine, IDIS-CIMUS, University of Santiago de Compostela, 15782 Santiago de Compostela, Spain. Fernández-Pombo A; UETeM-Molecular Pathology Group, Department of Psychiatry, Radiology, Public Health, Nursing and Medicine, IDIS-CIMUS, University of Santiago de Compostela, 15782 Santiago de Compostela, Spain.; Division of Endocrinology and Nutrition, University Clinical Hospital of Santiago de Compostela, 15706 Santiago de Compostela, Spain. Cobelo-Gómez S; UETeM-Molecular Pathology Group, Department of Psychiatry, Radiology, Public Health, Nursing and Medicine, IDIS-CIMUS, University of Santiago de Compostela, 15782 Santiago de Compostela, Spain. Hermida-Ameijeiras Á; UETeM-Molecular Pathology Group, Department of Psychiatry, Radiology, Public Health, Nursing and Medicine, IDIS-CIMUS, University of Santiago de Compostela, 15782 Santiago de Compostela, Spain.; Division of Internal Medicine, University Clinical Hospital of Santiago de Compostela, 15706 Santiago de Compostela, Spain. Alarcón-Martínez H; Department of Pediatric Neurology, Hospital Virgen de la Arrixaca and IMIB-Arrixaca, CIBERER-ISCIII, 28029 Madrid, Spain. Domingo-Jiménez R; Department of Pediatric Neurology, Hospital Virgen de la Arrixaca and IMIB-Arrixaca, CIBERER-ISCIII, 28029 Madrid, Spain. Ruíz Riquelme AI; German Center for Neurodegenerative Diseases (DZNE), 72076 Tübingen, Germany. RequenaJR; Prion Laboratory, CIMUS Biomedical Research Institute, University of Santiago de Compostela-IDIS, Santiago de Compostela, 15782 Santiago de Compostela, Spain. Araújo-Vilar D; UETeM-Molecular Pathology Group, Department of Psychiatry, Radiology, Public Health, Nursing and Medicine, IDIS-CIMUS, University of Santiago de Compostela, 15782 Santiago de Compostela, Spain.; Division of Endocrinology and Nutrition, University Clinical Hospital of Santiago de Compostela, 15706 Santiago de Compostela, Spain.
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Źródło:
Journal of clinical medicine [J Clin Med] 2021 Apr 01; Vol. 10 (7). Date of Electronic Publication: 2021 Apr 01.
Pharmacological inactivation of the prion protein by targeting a folding intermediate.
Autorzy:
Spagnolli G; Department of Cellular, Computational and Integrative Biology, University of Trento, 38123, Povo, TN, Italy.; Dulbecco Telethon Institute, University of Trento, 38123, Povo, TN, Italy. Massignan T; Department of Cellular, Computational and Integrative Biology, University of Trento, 38123, Povo, TN, Italy.; Dulbecco Telethon Institute, University of Trento, 38123, Povo, TN, Italy.; Sibylla Biotech SRL, 37121, Verona, VR, Italy. Astolfi A; Department of Pharmaceutical Sciences, University of Perugia, 06123, Perugia, PG, Italy. Biggi S; Department of Cellular, Computational and Integrative Biology, University of Trento, 38123, Povo, TN, Italy.; Dulbecco Telethon Institute, University of Trento, 38123, Povo, TN, Italy. Rigoli M; Department of Physics, University of Trento, Povo, Trento, TN, Italy. Brunelli P; Department of Cellular, Computational and Integrative Biology, University of Trento, 38123, Povo, TN, Italy.; Dulbecco Telethon Institute, University of Trento, 38123, Povo, TN, Italy. Libergoli M; Department of Cellular, Computational and Integrative Biology, University of Trento, 38123, Povo, TN, Italy.; Dulbecco Telethon Institute, University of Trento, 38123, Povo, TN, Italy. Ianeselli A; Department of Cellular, Computational and Integrative Biology, University of Trento, 38123, Povo, TN, Italy.; Dulbecco Telethon Institute, University of Trento, 38123, Povo, TN, Italy. Orioli S; Department of Physics, University of Trento, Povo, Trento, TN, Italy.; INFN-TIFPA, University of Trento, Povo, Trento, TN, Italy. Boldrini A; Department of Cellular, Computational and Integrative Biology, University of Trento, 38123, Povo, TN, Italy.; Sibylla Biotech SRL, 37121, Verona, VR, Italy. Terruzzi L; Department of Cellular, Computational and Integrative Biology, University of Trento, 38123, Povo, TN, Italy.; Sibylla Biotech SRL, 37121, Verona, VR, Italy. Bonaldo V; Department of Cellular, Computational and Integrative Biology, University of Trento, 38123, Povo, TN, Italy.; Dulbecco Telethon Institute, University of Trento, 38123, Povo, TN, Italy. Maietta G; Department of Cellular, Computational and Integrative Biology, University of Trento, 38123, Povo, TN, Italy.; Dulbecco Telethon Institute, University of Trento, 38123, Povo, TN, Italy. Lorenzo NL; CIMUS Biomedical Research Institute, University of Santiago de Compostela-IDIS, Santiago de Compostela, Spain. Fernandez LC; CIMUS Biomedical Research Institute, University of Santiago de Compostela-IDIS, Santiago de Compostela, Spain. Codeseira YB; CIMUS Biomedical Research Institute, University of Santiago de Compostela-IDIS, Santiago de Compostela, Spain. Tosatto L; Institute of Biophysics, National Council of Research, 38123 Povo, Trento, TN, Italy. Linsenmeier L; Institute of Neuropathology, University Medical Center Hamburg-Eppendorf, 20246, Hamburg, Germany. Vignoli B; Department of Physics, University of Trento, Povo, Trento, TN, Italy. Petris G; Department of Cellular, Computational and Integrative Biology, University of Trento, 38123, Povo, TN, Italy. Gasparotto D; Department of Cellular, Computational and Integrative Biology, University of Trento, 38123, Povo, TN, Italy.; Dulbecco Telethon Institute, University of Trento, 38123, Povo, TN, Italy. Pennuto M; Department of Biomedical Sciences (DBS), University of Padova, 35131, Padova, Italy.; Veneto Institute of Molecular Medicine (VIMM), 35129, Padova, Italy. Guella G; Department of Physics, University of Trento, Povo, Trento, TN, Italy. Canossa M; Department of Cellular, Computational and Integrative Biology, University of Trento, 38123, Povo, TN, Italy. Altmeppen HC; Institute of Neuropathology, University Medical Center Hamburg-Eppendorf, 20246, Hamburg, Germany. Lolli G; Department of Cellular, Computational and Integrative Biology, University of Trento, 38123, Povo, TN, Italy. Biressi S; Department of Cellular, Computational and Integrative Biology, University of Trento, 38123, Povo, TN, Italy.; Dulbecco Telethon Institute, University of Trento, 38123, Povo, TN, Italy. Pastor MM; RIAIDT, University of Santiago de Compostela-IDIS, Santiago de Compostela, Spain. RequenaJR; CIMUS Biomedical Research Institute, University of Santiago de Compostela-IDIS, Santiago de Compostela, Spain. Mancini I; Department of Physics, University of Trento, Povo, Trento, TN, Italy. Barreca ML; Department of Pharmaceutical Sciences, University of Perugia, 06123, Perugia, PG, Italy. . Faccioli P; Department of Physics, University of Trento, Povo, Trento, TN, Italy. .; INFN-TIFPA, University of Trento, Povo, Trento, TN, Italy. . Biasini E; Department of Cellular, Computational and Integrative Biology, University of Trento, 38123, Povo, TN, Italy. .; Dulbecco Telethon Institute, University of Trento, 38123, Povo, TN, Italy. .
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Źródło:
Communications biology [Commun Biol] 2021 Jan 12; Vol. 4 (1), pp. 62. Date of Electronic Publication: 2021 Jan 12.
Typ publikacji:
Journal Article; Research Support, Non-U.S. Gov't; Video-Audio Media
All-atom simulation of the HET-s prion replication.
Autorzy:
Terruzzi L; Sibylla Biotech SRL, Verona, Italy.; Department of Cellular, Computational and Integrative Biology (CIBIO), University of Trento, Povo, Trento, Italy. Spagnolli G; Department of Cellular, Computational and Integrative Biology (CIBIO), University of Trento, Povo, Trento, Italy.; Dulbecco Telethon Institute, University of Trento, Povo, Trento, Italy. Boldrini A; Sibylla Biotech SRL, Verona, Italy.; Department of Cellular, Computational and Integrative Biology (CIBIO), University of Trento, Povo, Trento, Italy. RequenaJR; CIMUS Biomedical Research Institute & Department of Medical Sciences, University of Santiago de Compostela-IDIS, Spain. Biasini E; Department of Cellular, Computational and Integrative Biology (CIBIO), University of Trento, Povo, Trento, Italy.; Dulbecco Telethon Institute, University of Trento, Povo, Trento, Italy. Faccioli P; Department of Physics, University of Trento, Povo, Trento, Italy.; INFN-TIFPA, University of Trento, Povo, Trento, Italy.
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Źródło:
PLoS computational biology [PLoS Comput Biol] 2020 Sep 18; Vol. 16 (9), pp. e1007922. Date of Electronic Publication: 2020 Sep 18 (Print Publication: 2020).
RequenaJR; CIMUS Biomedical Research Institute & Department of Medical Sciences, University of Santiago de Compostela-IDIS, Santiago de Compostela, Spain.
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Źródło:
PLoS biology [PLoS Biol] 2020 Jun 25; Vol. 18 (6), pp. e3000754. Date of Electronic Publication: 2020 Jun 25 (Print Publication: 2020).
Typ publikacji:
Journal Article; Research Support, Non-U.S. Gov't; Comment
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