PrPSc Prions: State of the Art
Prion diseases, or transmissible spongiform encephalopathies (TSEs), are a group of fatal and transmissible neurodegenerative disorders characterized by long incubation periods, misfolded prion protein (PrP) deposition, and usually spongiform vacuolation. These devastating diseases affect many mamma...
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MDPI - Multidisciplinary Digital Publishing Institute
2021
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| _version_ | 1869518182000099328 |
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| author | Jesús R. Requena (Ed.) Joaquín Castilla (Ed.) |
| author_browse | Jesús R. Requena (Ed.) Joaquín Castilla (Ed.) |
| author_facet | Jesús R. Requena (Ed.) Joaquín Castilla (Ed.) |
| author_sort | Jesús R. Requena (Ed.) |
| collection | Directory of Open Access Books |
| description | Prion diseases, or transmissible spongiform encephalopathies (TSEs), are a group of fatal and transmissible neurodegenerative disorders characterized by long incubation periods, misfolded prion protein (PrP) deposition, and usually spongiform vacuolation. These devastating diseases affect many mammals, with the best known examples being Creutzfeldt–Jakob disease (CJD), fatal familial insomnia (FFI), or Kuru in humans; and scrapie in sheep, bovine spongiform encephalopathy (BSE) in cattle, and chronic wasting disease (CWD) in cervids. Despite major achievements in research of TSEs, there are still many unresolved key issues that hamper the development of effective therapies. However, the last decade has been particularly prolific in advances in the prion field. Among others, prion propagation in vitro has been achieved, leading to new diagnostic methods; the basic architecture of infectious prions has been deciphered; new prion disease types have been described in humans and other animals; and prion disorders have emerged in places that had not previously reported the disorders, as is the case for CWD in Europe. This Special Issue will focus on the state of the art of our knowledge of PrPSc: on what we know about its structure and propagation, the basis of strains and transmission barriers, the mechanisms of PrPSc toxicity, the possible function of PrPSc’s properly folded precursor, PrPC and its evolutionary history, and recent technical breakthroughs in diagnostics and therapy development among other key aspects of PrPSc prion biology. |
| format | Online |
| id | doab-20.500.12854ir-57294 |
| institution | Directory of Open Access Books |
| language | eng |
| publishDate | 2021 |
| publishDateRange | 2021 |
| publishDateSort | 2021 |
| publisher | MDPI - Multidisciplinary Digital Publishing Institute |
| publisherStr | MDPI - Multidisciplinary Digital Publishing Institute |
| record_format | ojs |
| spelling | doab-20.500.12854ir-572942024-03-30T23:22:15Z PrPSc Prions: State of the Art Jesús R. Requena (Ed.) Joaquín Castilla (Ed.) R5-920 QH301-705.5 Prion propagation Therapy Scrapie Chronic wasting disease Gerstmann-Sträussler-Scheinker disease GSS Creutzfeldt-Jakob disease Bovine spongiform encephalopathy Prion structure CWD BSE Prions Therapeutics Prion diseases VPSPr Variably protease-sensitive prionopathy thema EDItEUR::M Medicine and Nursing Prion diseases, or transmissible spongiform encephalopathies (TSEs), are a group of fatal and transmissible neurodegenerative disorders characterized by long incubation periods, misfolded prion protein (PrP) deposition, and usually spongiform vacuolation. These devastating diseases affect many mammals, with the best known examples being Creutzfeldt–Jakob disease (CJD), fatal familial insomnia (FFI), or Kuru in humans; and scrapie in sheep, bovine spongiform encephalopathy (BSE) in cattle, and chronic wasting disease (CWD) in cervids. Despite major achievements in research of TSEs, there are still many unresolved key issues that hamper the development of effective therapies. However, the last decade has been particularly prolific in advances in the prion field. Among others, prion propagation in vitro has been achieved, leading to new diagnostic methods; the basic architecture of infectious prions has been deciphered; new prion disease types have been described in humans and other animals; and prion disorders have emerged in places that had not previously reported the disorders, as is the case for CWD in Europe. This Special Issue will focus on the state of the art of our knowledge of PrPSc: on what we know about its structure and propagation, the basis of strains and transmission barriers, the mechanisms of PrPSc toxicity, the possible function of PrPSc’s properly folded precursor, PrPC and its evolutionary history, and recent technical breakthroughs in diagnostics and therapy development among other key aspects of PrPSc prion biology. 2021-02-12T00:20:05Z 2021-02-12T00:20:05Z 2018-11-07 10:54:14 2018 book 29477 9783038973096 9783038973089 https://directory.doabooks.org/handle/20.500.12854/57294 eng image/jpeg Attribution-NonCommercial-NoDerivatives 4.0 International https://play.google.com/books/publish/a/14935057684283403269#details/ISBN:9783038973089 https://www.mdpi.com/books/pdfview/book/849 https://www.mdpi.com/books/pdfview/book/849 MDPI - Multidisciplinary Digital Publishing Institute 10.3390/books978-3-03897-309-6 10.3390/books978-3-03897-309-6 46cabcaa-dd94-4bfe-87b4-55023c1b36d0 9783038973096 9783038973089 210 open access |
| spellingShingle | R5-920 QH301-705.5 Prion propagation Therapy Scrapie Chronic wasting disease Gerstmann-Sträussler-Scheinker disease GSS Creutzfeldt-Jakob disease Bovine spongiform encephalopathy Prion structure CWD BSE Prions Therapeutics Prion diseases VPSPr Variably protease-sensitive prionopathy thema EDItEUR::M Medicine and Nursing Jesús R. Requena (Ed.) Joaquín Castilla (Ed.) PrPSc Prions: State of the Art |
| title | PrPSc Prions: State of the Art |
| title_full | PrPSc Prions: State of the Art |
| title_fullStr | PrPSc Prions: State of the Art |
| title_full_unstemmed | PrPSc Prions: State of the Art |
| title_short | PrPSc Prions: State of the Art |
| title_sort | prpsc prions state of the art |
| topic | R5-920 QH301-705.5 Prion propagation Therapy Scrapie Chronic wasting disease Gerstmann-Sträussler-Scheinker disease GSS Creutzfeldt-Jakob disease Bovine spongiform encephalopathy Prion structure CWD BSE Prions Therapeutics Prion diseases VPSPr Variably protease-sensitive prionopathy thema EDItEUR::M Medicine and Nursing |
| topic_facet | R5-920 QH301-705.5 Prion propagation Therapy Scrapie Chronic wasting disease Gerstmann-Sträussler-Scheinker disease GSS Creutzfeldt-Jakob disease Bovine spongiform encephalopathy Prion structure CWD BSE Prions Therapeutics Prion diseases VPSPr Variably protease-sensitive prionopathy thema EDItEUR::M Medicine and Nursing |
| url | 29477 |
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