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The Expanding Universe of Prion Diseases
Prions cause fatal and transmissible neurodegenerative disease. These etiological infectious agents are formed in greater part from a misfolded cell-surface protein called PrP(C). Several mammalian species are affected by the diseases, and in the case of “mad cow disease” (BSE) the agent has a tropi...
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Formato: | Texto |
Lenguaje: | English |
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Public Library of Science
2006
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Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC1434791/ https://www.ncbi.nlm.nih.gov/pubmed/16609731 http://dx.doi.org/10.1371/journal.ppat.0020026 |
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author | Watts, Joel C Balachandran, Aru Westaway, David |
author_facet | Watts, Joel C Balachandran, Aru Westaway, David |
author_sort | Watts, Joel C |
collection | PubMed |
description | Prions cause fatal and transmissible neurodegenerative disease. These etiological infectious agents are formed in greater part from a misfolded cell-surface protein called PrP(C). Several mammalian species are affected by the diseases, and in the case of “mad cow disease” (BSE) the agent has a tropism for humans, with negative consequences for agribusiness and public health. Unfortunately, the known universe of prion diseases is expanding. At least four novel prion diseases—including human diseases variant Creutzfeldt-Jakob disease (vCJD) and sporadic fatal insomnia (sFI), bovine amyloidotic spongiform encephalopathy (BASE), and Nor98 of sheep—have been identified in the last ten years, and chronic wasting disease (CWD) of North American deer (Odocoileus Specis) and Rocky Mountain elk (Cervus elaphus nelsoni) is undergoing a dramatic spread across North America. While amplification (BSE) and dissemination (CWD, commercial sourcing of cervids from the wild and movement of farmed elk) can be attributed to human activity, the origins of emergent prion diseases cannot always be laid at the door of humankind. Instead, the continued appearance of new outbreaks in the form of “sporadic” disease may be an inevitable outcome in a situation where the replicating pathogen is host-encoded. |
format | Text |
id | pubmed-1434791 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2006 |
publisher | Public Library of Science |
record_format | MEDLINE/PubMed |
spelling | pubmed-14347912006-04-10 The Expanding Universe of Prion Diseases Watts, Joel C Balachandran, Aru Westaway, David PLoS Pathog Review Prions cause fatal and transmissible neurodegenerative disease. These etiological infectious agents are formed in greater part from a misfolded cell-surface protein called PrP(C). Several mammalian species are affected by the diseases, and in the case of “mad cow disease” (BSE) the agent has a tropism for humans, with negative consequences for agribusiness and public health. Unfortunately, the known universe of prion diseases is expanding. At least four novel prion diseases—including human diseases variant Creutzfeldt-Jakob disease (vCJD) and sporadic fatal insomnia (sFI), bovine amyloidotic spongiform encephalopathy (BASE), and Nor98 of sheep—have been identified in the last ten years, and chronic wasting disease (CWD) of North American deer (Odocoileus Specis) and Rocky Mountain elk (Cervus elaphus nelsoni) is undergoing a dramatic spread across North America. While amplification (BSE) and dissemination (CWD, commercial sourcing of cervids from the wild and movement of farmed elk) can be attributed to human activity, the origins of emergent prion diseases cannot always be laid at the door of humankind. Instead, the continued appearance of new outbreaks in the form of “sporadic” disease may be an inevitable outcome in a situation where the replicating pathogen is host-encoded. Public Library of Science 2006-03 2006-03-31 /pmc/articles/PMC1434791/ /pubmed/16609731 http://dx.doi.org/10.1371/journal.ppat.0020026 Text en © 2006 Watts et al. http://creativecommons.org/licenses/by/4.0/ This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are properly credited. |
spellingShingle | Review Watts, Joel C Balachandran, Aru Westaway, David The Expanding Universe of Prion Diseases |
title | The Expanding Universe of Prion Diseases |
title_full | The Expanding Universe of Prion Diseases |
title_fullStr | The Expanding Universe of Prion Diseases |
title_full_unstemmed | The Expanding Universe of Prion Diseases |
title_short | The Expanding Universe of Prion Diseases |
title_sort | expanding universe of prion diseases |
topic | Review |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC1434791/ https://www.ncbi.nlm.nih.gov/pubmed/16609731 http://dx.doi.org/10.1371/journal.ppat.0020026 |
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