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Prion Volume 6, 2012 - Issue 2
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Mini Review

Molecular pathogenesis of sporadic prion diseases in man

Jiri G. Safar Departments of Pathology and Neurology; National Prion Disease Surveillance Center; School of Medicine; Case Western Reserve University; Cleveland, OH USA
Pages 108-115 | Received 03 Oct 2011, Accepted 04 Nov 2011, Published online: 01 Apr 2012

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Mark Cohen, Brian Appleby & Jiri G. Safar. (2016) Distinct prion-like strains of amyloid beta implicated in phenotypic diversity of Alzheimer's disease. Prion 10:1, pages 9-17.
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Kuntaek Lim, Su Yeon Kim, Byoungsub Lee, Christiane Segarra, Sungmin Kang, Youngran Ju, Mary Jo Schmerr, Joliette Coste, Sang Yun Kim, Takashi Yokoyama & Seong Soo A An. (2015) Magnetic microparticle-based multimer detection system for the detection of prion oligomers in sheep. International Journal of Nanomedicine 10:sup1, pages 241-250.
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M Enamul Kabir & Jiri G Safar. (2014) Implications of prion adaptation and evolution paradigm for human neurodegenerative diseases. Prion 8:1, pages 111-116.
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Lenka Hromadkova, M. Khursheed Siddiqi, He Liu & Jiri G. Safar. 2023. Prions and Diseases. Prions and Diseases 123 145 .
Lenka Hromadkova, Mohammad Khursheed Siddiqi, He Liu & Jiri G. Safar. (2022) Populations of Tau Conformers Drive Prion-like Strain Effects in Alzheimer’s Disease and Related Dementias. Cells 11:19, pages 2997.
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Hasier Eraña, Beatriz San Millán, Carlos M. Díaz-Domínguez, Jorge M. Charco, Rosa Rodríguez, Irene Viéitez, Arrate Pereda, Rosa Yañez, Mariví Geijo, Carmen Navarro, Guiomar Perez de Nanclares, Susana Teijeira & Joaquín Castilla. (2022) Description of the first Spanish case of Gerstmann–Sträussler–Scheinker disease with A117V variant: clinical, histopathological and biochemical characterization. Journal of Neurology 269:8, pages 4253-4263.
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Miren Altuna, Iñigo Ruiz, María Victoria Zelaya & Maite Mendioroz. (2022) Role of Biomarkers for the Diagnosis of Prion Diseases: A Narrative Review. Medicina 58:4, pages 473.
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Federico Angelo Cazzaniga, Edoardo Bistaffa, Chiara Maria Giulia De Luca, Sara Maria Portaleone, Marcella Catania, Veronica Redaelli, Irene Tramacere, Giuseppe Bufano, Martina Rossi, Paola Caroppo, Anna Rita Giovagnoli, Pietro Tiraboschi, Giuseppe Di Fede, Roberto Eleopra, Grazia Devigili, Antonio Emanuele Elia, Roberto Cilia, Michele Fiorini, Matilde Bongianni, Giulia Salzano, Luigi Celauro, Federico Giuseppe Quarta, Angela Mammana, Giuseppe Legname, Fabrizio Tagliavini, Piero Parchi, Gianluigi Zanusso, Giorgio Giaccone & Fabio Moda. (2022) PMCA-Based Detection of Prions in the Olfactory Mucosa of Patients With Sporadic Creutzfeldt–Jakob Disease. Frontiers in Aging Neuroscience 14.
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Chae Kim, Tracy Haldiman, Sang-Gyun Kang, Lenka Hromadkova, Zhuang Zhuang Han, Wei ChenFrances Lissemore, Alan Lerner, Rohan de Silva, Mark L. CohenDavid Westaway & Jiri G. Safar. (2022) Distinct populations of highly potent TAU seed conformers in rapidly progressing Alzheimer’s disease. Science Translational Medicine 14:626.
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Kevin M. Alexander, Isabel Morgado & Ronglih Liao. 2022. Biochemistry of Apoptosis and Autophagy. Biochemistry of Apoptosis and Autophagy 219 237 .
He Liu, Chae Kim, Tracy Haldiman, Christina J. Sigurdson, Sofie Nyström, K. Peter R. Nilsson, Mark L. Cohen, Thomas Wisniewski, Per Hammarström & Jiri G. Safar. (2021) Distinct conformers of amyloid beta accumulate in the neocortex of patients with rapidly progressive Alzheimer's disease. Journal of Biological Chemistry 297:5, pages 101267.
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Federico Angelo Cazzaniga, Edoardo Bistaffa, Chiara Maria Giulia De Luca, Giuseppe Bufano, Antonio Indaco, Giorgio Giaccone & Fabio Moda. (2021) Sporadic Creutzfeldt-Jakob disease: Real-Time Quaking Induced Conversion (RT-QuIC) assay represents a major diagnostic advance. European Journal of Histochemistry 65:s1.
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Mohammad Khursheed Siddiqi, Chae Kim, Tracy Haldiman, Miroslava Kacirova, Benlian Wang, Jen Bohon, Mark R. Chance, Janna Kiselar & Jiri G. Safar. (2021) Structurally distinct external solvent-exposed domains drive replication of major human prions. PLOS Pathogens 17:6, pages e1009642.
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Neil Watson, Jean-Philippe Brandel, Alison Green, Peter Hermann, Anna Ladogana, Terri Lindsay, Janet Mackenzie, Maurizio Pocchiari, Colin Smith, Inga Zerr & Suvankar Pal. (2021) The importance of ongoing international surveillance for Creutzfeldt–Jakob disease. Nature Reviews Neurology 17:6, pages 362-379.
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Nathalie Daude, Chae Kim, Sang-Gyun Kang, Ghazaleh Eskandari-Sedighi, Tracy Haldiman, Jing Yang, Shelaine C. Fleck, Erik Gomez-Cardona, Zhuang Zhuang Han, Sergi Borrego-Ecija, Serene Wohlgemuth, Olivier Julien, Holger Wille, Laura Molina-Porcel, Ellen Gelpi, Jiri G. Safar & David Westaway. (2020) Diverse, evolving conformer populations drive distinct phenotypes in frontotemporal lobar degeneration caused by the same MAPT-P301L mutation. Acta Neuropathologica 139:6, pages 1045-1070.
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Luis E. Escobar, Sandra Pritzkow, Steven N. Winter, Daniel A. Grear, Megan S. Kirchgessner, Ernesto Dominguez‐Villegas, Gustavo Machado, A. Townsend Peterson & Claudio Soto. (2019) The ecology of chronic wasting disease in wildlife. Biological Reviews 95:2, pages 393-408.
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Camilo Duque Velásquez, Chae Kim, Tracy Haldiman, Chiye Kim, Allen Herbst, Judd Aiken, Jiri G. Safar & Debbie McKenzie. (2020) Chronic wasting disease (CWD) prion strains evolve via adaptive diversification of conformers in hosts expressing prion protein polymorphisms. Journal of Biological Chemistry 295:15, pages 4985-5001.
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Takuto Shimizu, Yoshimichi Kozuka, Mayumi Kusano, Masaki Nagane, Tadashi Yamashita & Naomi Hachiya. (2020) PrP (122–139) is a covert mitochondrial targeting signal of prion protein and it specifically triggers the perinuclear clustering of mitochondria in neuronal culture cells. Biochemical and Biophysical Research Communications 524:2, pages 301-307.
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Johannes Manjrekar & Hiral Shah. (2020) Protein-based inheritance. Seminars in Cell & Developmental Biology 97, pages 138-155.
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Andrea N. Killian, Sarah C. Miller & Justin K. Hines. (2019) Impact of Amyloid Polymorphism on Prion-Chaperone Interactions in Yeast. Viruses 11:4, pages 349.
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Saima Zafar, Neelam Younas, Mohsin Shafiq & Inga Zerr. 2019. Prions - Some Physiological and Pathophysiological Aspects. Prions - Some Physiological and Pathophysiological Aspects.
Timothy E. Yap, Shiama I. Balendra, Melanie T. Almonte & M. Francesca Cordeiro. (2019) Retinal correlates of neurological disorders. Therapeutic Advances in Chronic Disease 10, pages 204062231988220.
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Jules J. Berman. 2019. Taxonomic Guide to Infectious Diseases. Taxonomic Guide to Infectious Diseases 321 365 .
David Seynnaeve, Mara Vecchio, Gernot Fruhmann, Joke Verelst, Melody Cools, Jimmy Beckers, Daniel Mulvihill, Joris Winderickx & Vanessa Franssens. (2018) Recent Insights on Alzheimer’s Disease Originating from Yeast Models. International Journal of Molecular Sciences 19:7, pages 1947.
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Jonathan D. F. Wadsworth, Gary Adamson, Susan Joiner, Lara Brock, Caroline Powell, Jacqueline M. Linehan, Jonathan A. Beck, Sebastian Brandner, Simon Mead & John Collinge. 2017. Prions. Prions 311 346 .
Susan F. Godsave, Peter J. Peters & Holger Wille. (2015) Subcellular distribution of the prion protein in sickness and in health. Virus Research 207, pages 136-145.
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Robert B. Petersen, Frances M. Lissemore, Brian Appleby, Neelum Aggarwal, Richard Boyatzis, Gemma Casadesus, Jeff Cummings, Anthony Jack, George Perry, Jiri Safar, Martha Sajatovic, Witold K. Surewicz, Yanming Wang, Peter Whitehouse & Alan Lerner. (2015) From Neurodegeneration to Brain Health: An Integrated Approach. Journal of Alzheimer's Disease 46:1, pages 271-283.
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Mark L. Cohen, Chae Kim, Tracy Haldiman, Mohamed ElHag, Prachi Mehndiratta, Termsarasab Pichet, Frances Lissemore, Michelle Shea, Yvonne Cohen, Wei Chen, Janis Blevins, Brian S. Appleby, Krystyna Surewicz, Witold K. Surewicz, Martha Sajatovic, Curtis Tatsuoka, Shulin Zhang, Ping Mayo, Mariusz Butkiewicz, Jonathan L. Haines, Alan J. Lerner & Jiri G. Safar. (2015) Rapidly progressive Alzheimer’s disease features distinct structures of amyloid-β. Brain 138:4, pages 1009-1022.
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Vladimir N. Uversky. (2015) Intrinsically disordered proteins and their (disordered) proteomes in neurodegenerative disorders. Frontiers in Aging Neuroscience 7.
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Frank O. Bastian. (2015) Is Alzheimer's Disease Infectious?<br><i>Relative to the CJD Bacterial Infection Model of Neurodegeneration</i>. AIMS Neuroscience 2:4, pages 240-258.
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Vladimir N. Uversky. (2014) Wrecked regulation of intrinsically disordered proteins in diseases: pathogenicity of deregulated regulators. Frontiers in Molecular Biosciences 1.
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Marco Sandri & Jeffrey Robbins. (2014) Proteotoxicity: An underappreciated pathology in cardiac disease. Journal of Molecular and Cellular Cardiology 71, pages 3-10.
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Kevin C. Stein & Heather L. True. (2014) Extensive Diversity of Prion Strains Is Defined by Differential Chaperone Interactions and Distinct Amyloidogenic Regions. PLoS Genetics 10:5, pages e1004337.
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Laura Westergard & Heather L. True. (2014) Extracellular environment modulates the formation and propagation of particular amyloid structures. Molecular Microbiology 92:4, pages 698-715.
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Vincent J. Huang, Kevin C. Stein & Heather L. True. (2013) Spontaneous Variants of the [RNQ+] Prion in Yeast Demonstrate the Extensive Conformational Diversity Possible with Prion Proteins. PLoS ONE 8:10, pages e79582.
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Tracy Haldiman, Chae Kim, Yvonne Cohen, Wei Chen, Janis Blevins, Liuting Qing, Mark L. Cohen, Jan Langeveld, Glenn C. Telling, Qingzhong Kong & Jiri G. Safar. (2013) Co-existence of Distinct Prion Types Enables Conformational Evolution of Human PrP Sc by Competitive Selection . Journal of Biological Chemistry 288:41, pages 29846-29861.
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Jiri G. Safar. 2013. Prions and Diseases. Prions and Diseases 161 179 .

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