Human prion diseases and the prion protein – what is the current state of knowledge?
Prion diseases and the prion protein are only partially understood so far in many aspects. This explains the continued research on this topic, calling for an overview on the current state of knowledge. The main objective of the present review article is to provide a comprehensive up-to-date presenta...
| Main Authors: | , , |
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| Format: | Article |
| Language: | English |
| Published: |
De Gruyter
2023-10-01
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| Series: | Translational Neuroscience |
| Subjects: | |
| Online Access: | https://doi.org/10.1515/tnsci-2022-0315 |
| _version_ | 1797653338779549696 |
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| author | Nafe Reinhold Arendt Christophe T. Hattingen Elke |
| author_facet | Nafe Reinhold Arendt Christophe T. Hattingen Elke |
| author_sort | Nafe Reinhold |
| collection | DOAJ |
| description | Prion diseases and the prion protein are only partially understood so far in many aspects. This explains the continued research on this topic, calling for an overview on the current state of knowledge. The main objective of the present review article is to provide a comprehensive up-to-date presentation of all major features of human prion diseases bridging the gap between basic research and clinical aspects. Starting with the prion protein, current insights concerning its physiological functions and the process of pathological conversion will be highlighted. Diagnostic, molecular, and clinical aspects of all human prion diseases will be discussed, including information concerning rare diseases like prion-associated amyloidoses and Huntington disease-like 1, as well as the question about a potential human threat due to the transmission of prions from prion diseases of other species such as chronic wasting disease. Finally, recent attempts to develop future therapeutic strategies will be addressed. |
| first_indexed | 2024-03-11T16:43:12Z |
| format | Article |
| id | doaj.art-4b467cf5c7a54ea283fbe27c0387d6ef |
| institution | Directory Open Access Journal |
| issn | 2081-6936 |
| language | English |
| last_indexed | 2024-03-11T16:43:12Z |
| publishDate | 2023-10-01 |
| publisher | De Gruyter |
| record_format | Article |
| series | Translational Neuroscience |
| spelling | doaj.art-4b467cf5c7a54ea283fbe27c0387d6ef2023-10-23T07:51:20ZengDe GruyterTranslational Neuroscience2081-69362023-10-01141608310.1515/tnsci-2022-0315Human prion diseases and the prion protein – what is the current state of knowledge?Nafe Reinhold0Arendt Christophe T.1Hattingen Elke2Department of Neuroradiology, Clinics of Johann Wolfgang-Goethe University, Schleusenweg 2-16, 60528Frankfurt am Main, GermanyDepartment of Neuroradiology, Clinics of Johann Wolfgang-Goethe University, Schleusenweg 2-16, 60528Frankfurt am Main, GermanyDepartment of Neuroradiology, Clinics of Johann Wolfgang-Goethe University, Schleusenweg 2-16, 60528Frankfurt am Main, GermanyPrion diseases and the prion protein are only partially understood so far in many aspects. This explains the continued research on this topic, calling for an overview on the current state of knowledge. The main objective of the present review article is to provide a comprehensive up-to-date presentation of all major features of human prion diseases bridging the gap between basic research and clinical aspects. Starting with the prion protein, current insights concerning its physiological functions and the process of pathological conversion will be highlighted. Diagnostic, molecular, and clinical aspects of all human prion diseases will be discussed, including information concerning rare diseases like prion-associated amyloidoses and Huntington disease-like 1, as well as the question about a potential human threat due to the transmission of prions from prion diseases of other species such as chronic wasting disease. Finally, recent attempts to develop future therapeutic strategies will be addressed.https://doi.org/10.1515/tnsci-2022-0315creutzfeldt–jakob diseasevariably protease-sensitive prionopathyfatal familial insomniagerstmann–sträussler–scheinker diseasekuru |
| spellingShingle | Nafe Reinhold Arendt Christophe T. Hattingen Elke Human prion diseases and the prion protein – what is the current state of knowledge? Translational Neuroscience creutzfeldt–jakob disease variably protease-sensitive prionopathy fatal familial insomnia gerstmann–sträussler–scheinker disease kuru |
| title | Human prion diseases and the prion protein – what is the current state of knowledge? |
| title_full | Human prion diseases and the prion protein – what is the current state of knowledge? |
| title_fullStr | Human prion diseases and the prion protein – what is the current state of knowledge? |
| title_full_unstemmed | Human prion diseases and the prion protein – what is the current state of knowledge? |
| title_short | Human prion diseases and the prion protein – what is the current state of knowledge? |
| title_sort | human prion diseases and the prion protein what is the current state of knowledge |
| topic | creutzfeldt–jakob disease variably protease-sensitive prionopathy fatal familial insomnia gerstmann–sträussler–scheinker disease kuru |
| url | https://doi.org/10.1515/tnsci-2022-0315 |
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