The architecture of prions: how understanding would provide new therapeutic insights
Compelling evidence from the last three decades clearly shows that transmissible spongiform encephalopathies (TSEs) develop as a result of a poorly understood misfolding event that converts the cellular prion protein (PrPC) to an isoform known as PrPSc which is aggregated, protease resistant and a...
| Main Authors: | , |
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| Format: | Article |
| Language: | English |
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SMW supporting association (Trägerverein Swiss Medical Weekly SMW)
2016-09-01
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| Series: | Swiss Medical Weekly |
| Subjects: | |
| Online Access: | https://www.smw.ch/index.php/smw/article/view/2225 |
| _version_ | 1811289166755921920 |
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| author | Hasier Eraña Joaquín Castilla |
| author_facet | Hasier Eraña Joaquín Castilla |
| author_sort | Hasier Eraña |
| collection | DOAJ |
| description |
Compelling evidence from the last three decades clearly shows that transmissible spongiform encephalopathies (TSEs) develop as a result of a poorly understood misfolding event that converts the cellular prion protein (PrPC) to an isoform known as PrPSc which is aggregated, protease resistant and able to impose its aberrant conformation onto PrPC, leading to its accumulation in the central nervous system. Despite all the knowledge gathered in more than thirty years of research and the general understanding of the pathological processes, the molecular mechanisms remain elusive, making it difficult to develop rational therapeutic strategies for this group of incurable diseases. In this review article, we give an overview of what is known about prion architecture and how the limited structural information available has been used in the quest for remedies for these devastating disorders.
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| first_indexed | 2024-04-13T03:50:05Z |
| format | Article |
| id | doaj.art-74157450f1c148c182f032bc9c414771 |
| institution | Directory Open Access Journal |
| issn | 1424-3997 |
| language | English |
| last_indexed | 2024-04-13T03:50:05Z |
| publishDate | 2016-09-01 |
| publisher | SMW supporting association (Trägerverein Swiss Medical Weekly SMW) |
| record_format | Article |
| series | Swiss Medical Weekly |
| spelling | doaj.art-74157450f1c148c182f032bc9c4147712022-12-22T03:03:50ZengSMW supporting association (Trägerverein Swiss Medical Weekly SMW)Swiss Medical Weekly1424-39972016-09-01146394010.4414/smw.2016.14354The architecture of prions: how understanding would provide new therapeutic insightsHasier ErañaJoaquín Castilla Compelling evidence from the last three decades clearly shows that transmissible spongiform encephalopathies (TSEs) develop as a result of a poorly understood misfolding event that converts the cellular prion protein (PrPC) to an isoform known as PrPSc which is aggregated, protease resistant and able to impose its aberrant conformation onto PrPC, leading to its accumulation in the central nervous system. Despite all the knowledge gathered in more than thirty years of research and the general understanding of the pathological processes, the molecular mechanisms remain elusive, making it difficult to develop rational therapeutic strategies for this group of incurable diseases. In this review article, we give an overview of what is known about prion architecture and how the limited structural information available has been used in the quest for remedies for these devastating disorders. https://www.smw.ch/index.php/smw/article/view/2225Misfolding proteinsprion diseasesTherapeutical approachesTransmissible Spongiform Encephalopathies (TSEs) |
| spellingShingle | Hasier Eraña Joaquín Castilla The architecture of prions: how understanding would provide new therapeutic insights Swiss Medical Weekly Misfolding proteins prion diseases Therapeutical approaches Transmissible Spongiform Encephalopathies (TSEs) |
| title | The architecture of prions: how understanding would provide new therapeutic insights |
| title_full | The architecture of prions: how understanding would provide new therapeutic insights |
| title_fullStr | The architecture of prions: how understanding would provide new therapeutic insights |
| title_full_unstemmed | The architecture of prions: how understanding would provide new therapeutic insights |
| title_short | The architecture of prions: how understanding would provide new therapeutic insights |
| title_sort | architecture of prions how understanding would provide new therapeutic insights |
| topic | Misfolding proteins prion diseases Therapeutical approaches Transmissible Spongiform Encephalopathies (TSEs) |
| url | https://www.smw.ch/index.php/smw/article/view/2225 |
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