{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,19]],"date-time":"2026-10-19T09:12:20Z","timestamp":1792401140795,"version":"4.4.0"},"reference-count":51,"publisher":"MDPI AG","issue":"1","license":[{"start":{"date-parts":[[2018,2,7]],"date-time":"2018-02-07T00:00:00Z","timestamp":1517961600000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"name":"Alberta Prion Research Institute","award":["APRI 201600012"],"award-info":[{"award-number":["APRI 201600012"]}]},{"name":"Alberta Prion Research Institute","award":["APRI 201600029"],"award-info":[{"award-number":["APRI 201600029"]}]},{"DOI":"10.13039\/501100002715","name":"Alberta Livestock and Meat Agency","doi-asserted-by":"publisher","award":["ALMA 2016A001R"],"award-info":[{"award-number":["ALMA 2016A001R"]}],"id":[{"id":"10.13039\/501100002715","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100000780","name":"European Commission","doi-asserted-by":"publisher","award":["FP7222887"],"award-info":[{"award-number":["FP7222887"]}],"id":[{"id":"10.13039\/501100000780","id-type":"DOI","asserted-by":"publisher"}]},{"name":"Spanish Ministry of Education (with FEDER funds)","award":["BFU2006-04588\/BMC"],"award-info":[{"award-number":["BFU2006-04588\/BMC"]}]},{"name":"Spanish Ministry of Economy (with FEDER funds)","award":["BFU2013-48436-C2-1-P"],"award-info":[{"award-number":["BFU2013-48436-C2-1-P"]}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Pathogens"],"abstract":"<jats:p>PrPSc (scrapie isoform of the prion protein) prions are the infectious agent behind diseases such as Creutzfeldt\u2013Jakob disease in humans, bovine spongiform encephalopathy in cattle, chronic wasting disease in cervids (deer, elk, moose, and reindeer), as well as goat and sheep scrapie. PrPSc is an alternatively folded variant of the cellular prion protein, PrPC, which is a regular, GPI-anchored protein that is present on the cell surface of neurons and other cell types. While the structure of PrPC is well studied, the structure of PrPSc resisted high-resolution determination due to its general insolubility and propensity to aggregate. Cryo-electron microscopy, X-ray fiber diffraction, and a variety of other approaches defined the structure of PrPSc as a four-rung \u03b2-solenoid. A high-resolution structure of PrPSc still remains to be solved, but the four-rung \u03b2-solenoid architecture provides a molecular framework for the autocatalytic propagation mechanism that gives rise to the alternative conformation of PrPSc. Here, we summarize the current knowledge regarding the structure of PrPSc and speculate about the molecular conversion mechanisms that leads from PrPC to PrPSc.<\/jats:p>","DOI":"10.3390\/pathogens7010020","type":"journal-article","created":{"date-parts":[[2018,2,7]],"date-time":"2018-02-07T12:20:29Z","timestamp":1518006029000},"page":"20","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":86,"title":["The Structure of PrPSc Prions"],"prefix":"10.3390","volume":"7","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-5102-8706","authenticated-orcid":false,"given":"Holger","family":"Wille","sequence":"first","affiliation":[{"name":"Centre for Prions and Protein Folding Diseases &amp; Department of Biochemistry, University of Alberta, Edmonton, AB T6G 2M8, Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jes\u00fas","family":"Requena","sequence":"additional","affiliation":[{"name":"CIMUS Biomedical Research Institute &amp; Department of Medical Sciences, University of Santiago de Compostela-IDIS, 15782 Santiago de Compostela, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2018,2,7]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"165","DOI":"10.1080\/19336896.2016.1175801","article-title":"The Priority position paper: Protecting Europe\u2019s food chain from prions","volume":"10","author":"Requena","year":"2016","journal-title":"Prion"},{"key":"ref_2","first-page":"1552","article-title":"Pathologie animale\u2014La maladie dite tremblante du mouton est-elle inoculable?","volume":"203","author":"Cuille","year":"1936","journal-title":"C. 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