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Open Access
Peer-reviewed
Research Article
A novel Gerstmann-Sträussler-Scheinker disease mutation defines a precursor for amyloidogenic 8 kDa PrP fragments and reveals N-terminal structural changes shared by other GSS alleles
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Robert C. C. Mercer,
Roles Conceptualization, Investigation, Writing – original draft, Writing – review & editing
Affiliations Centre for Prions and Protein Folding Diseases, University of Alberta, Edmonton, Alberta, Canada, Department of Medicine (Neurology), University of Alberta, Edmonton, Alberta, Canada
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Nathalie Daude,
Roles Conceptualization, Investigation
Affiliation Centre for Prions and Protein Folding Diseases, University of Alberta, Edmonton, Alberta, Canada
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Lyudmyla Dorosh,
Roles Conceptualization, Investigation
Affiliations National Research Council of Canada, Edmonton, Alberta, Canada, Department of Electrical and Computer Engineering, University of Alberta, Edmonton, Alberta, Canada
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Ze-Lin Fu,
Roles Investigation
Affiliations Centre for Prions and Protein Folding Diseases, University of Alberta, Edmonton, Alberta, Canada, Department of Biochemistry, University of Alberta, Edmonton, Alberta, Canada
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Charles E. Mays,
Roles Investigation
Affiliation Centre for Prions and Protein Folding Diseases, University of Alberta, Edmonton, Alberta, Canada
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Hristina Gapeshina,
Roles Investigation
Affiliation Centre for Prions and Protein Folding Diseases, University of Alberta, Edmonton, Alberta, Canada
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Serene L. Wohlgemuth,
Roles Conceptualization, Investigation
Affiliation Centre for Prions and Protein Folding Diseases, University of Alberta, Edmonton, Alberta, Canada
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Claudia Y. Acevedo-Morantes,
Roles Investigation
Affiliation Centre for Prions and Protein Folding Diseases, University of Alberta, Edmonton, Alberta, Canada
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Jing Yang,
Roles Investigation
Affiliation Centre for Prions and Protein Folding Diseases, University of Alberta, Edmonton, Alberta, Canada
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Neil R. Cashman,
Roles Resources
Affiliation Brain Research Centre, University of British Columbia, Vancouver, British Columbia, Canada
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Michael B. Coulthart,
Roles Resources
Affiliation Canadian Creutzfeldt-Jakob Disease Surveillance System, Centre for Foodborne, Environmental and Zoonotic Infectious Diseases, Public Health Agency of Canada, Ottawa, Ontario, Canada
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Dawn M. Pearson,
Roles Resources
Affiliation Department of Clinical Neurosciences, University of Calgary, Calgary, Alberta, Canada
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Jeffrey T. Joseph,
Roles Resources
Affiliation Hotchkiss Brain Institute and Calgary Laboratory Services, University of Calgary, Calgary, Alberta, Canada
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Holger Wille,
Roles Resources
Affiliations Centre for Prions and Protein Folding Diseases, University of Alberta, Edmonton, Alberta, Canada, Department of Biochemistry, University of Alberta, Edmonton, Alberta, Canada
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Jiri G. Safar,
Roles Resources
Affiliation Departments of Pathology and Neurology, School of Medicine Case Western Reserve University, Cleveland, Ohio, United States of America
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Gerard H. Jansen,
Roles Investigation, Resources
Affiliations Canadian Creutzfeldt-Jakob Disease Surveillance System, Centre for Foodborne, Environmental and Zoonotic Infectious Diseases, Public Health Agency of Canada, Ottawa, Ontario, Canada, Division of Anatomical Pathology, University of Ottawa, Ottawa, Ontario, Canada
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Maria Stepanova,
Roles Conceptualization, Resources
Affiliations National Research Council of Canada, Edmonton, Alberta, Canada, Department of Electrical and Computer Engineering, University of Alberta, Edmonton, Alberta, Canada
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Brian D. Sykes,
Roles Conceptualization, Resources
Affiliations Centre for Prions and Protein Folding Diseases, University of Alberta, Edmonton, Alberta, Canada, Department of Biochemistry, University of Alberta, Edmonton, Alberta, Canada
⨯ - [ ... ],
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David Westaway
Roles Conceptualization, Resources, Writing – original draft, Writing – review & editing
* E-mail: david.westaway@ualberta.ca
Affiliations Centre for Prions and Protein Folding Diseases, University of Alberta, Edmonton, Alberta, Canada, Department of Medicine (Neurology), University of Alberta, Edmonton, Alberta, Canada, Department of Biochemistry, University of Alberta, Edmonton, Alberta, Canada
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A novel Gerstmann-Sträussler-Scheinker disease mutation defines a precursor for amyloidogenic 8 kDa PrP fragments and reveals N-terminal structural changes shared by other GSS alleles
- Robert C. C. Mercer,
- Nathalie Daude,
- Lyudmyla Dorosh,
- Ze-Lin Fu,
- Charles E. Mays,
- Hristina Gapeshina,
- Serene L. Wohlgemuth,
- Claudia Y. Acevedo-Morantes,
- Jing Yang,
- Neil R. Cashman
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- Published: January 16, 2018
- https://doi.org/10.1371/journal.ppat.1006826