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 h...
| Main Authors: | , , |
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| Format: | Article |
| Language: | English |
| Published: |
Public Library of Science (PLoS)
2006-03-01
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| Series: | PLoS Pathogens |
| Online Access: | http://europepmc.org/articles/PMC1434791?pdf=render |
| _version_ | 1818096723064520704 |
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| author | Joel C Watts Aru Balachandran David Westaway |
| author_facet | Joel C Watts Aru Balachandran David Westaway |
| author_sort | Joel C Watts |
| collection | DOAJ |
| 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. |
| first_indexed | 2024-12-10T23:09:09Z |
| format | Article |
| id | doaj.art-59f18930c6384907afb4139ff9044181 |
| institution | Directory Open Access Journal |
| issn | 1553-7366 1553-7374 |
| language | English |
| last_indexed | 2024-12-10T23:09:09Z |
| publishDate | 2006-03-01 |
| publisher | Public Library of Science (PLoS) |
| record_format | Article |
| series | PLoS Pathogens |
| spelling | doaj.art-59f18930c6384907afb4139ff90441812022-12-22T01:29:59ZengPublic Library of Science (PLoS)PLoS Pathogens1553-73661553-73742006-03-0123e2610.1371/journal.ppat.0020026The expanding universe of prion diseases.Joel C WattsAru BalachandranDavid WestawayPrions 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.http://europepmc.org/articles/PMC1434791?pdf=render |
| spellingShingle | Joel C Watts Aru Balachandran David Westaway The expanding universe of prion diseases. PLoS Pathogens |
| 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 |
| url | http://europepmc.org/articles/PMC1434791?pdf=render |
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