Prion meeting 2023: implications of a growing field
The history of human prion diseases began with the original description, by Hans Gerhard Creutzfeldt and by Alfons Maria Jakob, of patients with a severe brain disease that included speech abnormalities, confusion, and myoclonus, in a disease that was then named Creutzfeldt Jakob disease (CJD). Late...
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Taylor & Francis Group
2024-12-01
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| Series: | Prion |
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| Online Access: | https://www.tandfonline.com/doi/10.1080/19336896.2024.2343535 |
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| author | Tiago F. Outeiro Tuane C. R. G. Vieira |
| author_facet | Tiago F. Outeiro Tuane C. R. G. Vieira |
| author_sort | Tiago F. Outeiro |
| collection | DOAJ |
| description | The history of human prion diseases began with the original description, by Hans Gerhard Creutzfeldt and by Alfons Maria Jakob, of patients with a severe brain disease that included speech abnormalities, confusion, and myoclonus, in a disease that was then named Creutzfeldt Jakob disease (CJD). Later, in Papua New Guinea, a disease characterized by trembling was identified, and given the name “Kuru”. Neuropathological examination of the brains from CJD and Kuru patients, and of brains of sheep with scrapie disease revealed significant similarities and suggested a possible common mode of infection that, at the time, was thought to derive from an unknown virus that caused slow infections. John Stanley Griffith hypothesized that the agent causing these diseases was “probably a protein without nucleic acid” and, in 1982, Stanley Prusiner reported the identification of a proteinaceous infectious particle (coining the term prion) that was resistant to inactivation methods that were at the time standard for nucleic acids, and identified PrP as the major protein component of the infectious agent in scrapie and in Creutzfeldt-Jakob disease, classifying this also as a prion disease. Interestingly, the prion concept had been previously expanded to yeast proteins capable of replicating their conformation, seeding their own aggregation and transmitting phenotypic information. The prion concept has been more recently expanded to refer to misfolded proteins that are capable of converting a normal form of a protein into an abnormal form. The quest to understand and treat prion diseases has united a specific research community around the topic, and regular meetings (Prion Meetings) have taken place over the years to enable discussions, train junior researchers, and inspire research in the field. |
| format | Article |
| id | doaj-art-421b126019d44c0aa4d4b4a3ca0f1ee2 |
| institution | Kabale University |
| issn | 1933-6896 1933-690X |
| language | English |
| publishDate | 2024-12-01 |
| publisher | Taylor & Francis Group |
| record_format | Article |
| series | Prion |
| spelling | doaj-art-421b126019d44c0aa4d4b4a3ca0f1ee22025-02-05T12:40:51ZengTaylor & Francis GroupPrion1933-68961933-690X2024-12-01181687110.1080/19336896.2024.2343535Prion meeting 2023: implications of a growing fieldTiago F. Outeiro0Tuane C. R. G. Vieira1Department of Experimental Neurodegeneration, Center for Biostructural Imaging of Neurodegeneration, University Medical Center Göttingen, Göttingen, GermanyInstitute of Medical Biochemistry Leopoldo de Meis and National Institute of Science and Technology for Structural Biology and Bioimaging, Federal University of Rio de Janeiro, Rio de Janeiro, BrazilThe history of human prion diseases began with the original description, by Hans Gerhard Creutzfeldt and by Alfons Maria Jakob, of patients with a severe brain disease that included speech abnormalities, confusion, and myoclonus, in a disease that was then named Creutzfeldt Jakob disease (CJD). Later, in Papua New Guinea, a disease characterized by trembling was identified, and given the name “Kuru”. Neuropathological examination of the brains from CJD and Kuru patients, and of brains of sheep with scrapie disease revealed significant similarities and suggested a possible common mode of infection that, at the time, was thought to derive from an unknown virus that caused slow infections. John Stanley Griffith hypothesized that the agent causing these diseases was “probably a protein without nucleic acid” and, in 1982, Stanley Prusiner reported the identification of a proteinaceous infectious particle (coining the term prion) that was resistant to inactivation methods that were at the time standard for nucleic acids, and identified PrP as the major protein component of the infectious agent in scrapie and in Creutzfeldt-Jakob disease, classifying this also as a prion disease. Interestingly, the prion concept had been previously expanded to yeast proteins capable of replicating their conformation, seeding their own aggregation and transmitting phenotypic information. The prion concept has been more recently expanded to refer to misfolded proteins that are capable of converting a normal form of a protein into an abnormal form. The quest to understand and treat prion diseases has united a specific research community around the topic, and regular meetings (Prion Meetings) have taken place over the years to enable discussions, train junior researchers, and inspire research in the field.https://www.tandfonline.com/doi/10.1080/19336896.2024.2343535Alpha-synucleinamyloidbeta-amyloidcancerneurodegenerationp53 |
| spellingShingle | Tiago F. Outeiro Tuane C. R. G. Vieira Prion meeting 2023: implications of a growing field Prion Alpha-synuclein amyloid beta-amyloid cancer neurodegeneration p53 |
| title | Prion meeting 2023: implications of a growing field |
| title_full | Prion meeting 2023: implications of a growing field |
| title_fullStr | Prion meeting 2023: implications of a growing field |
| title_full_unstemmed | Prion meeting 2023: implications of a growing field |
| title_short | Prion meeting 2023: implications of a growing field |
| title_sort | prion meeting 2023 implications of a growing field |
| topic | Alpha-synuclein amyloid beta-amyloid cancer neurodegeneration p53 |
| url | https://www.tandfonline.com/doi/10.1080/19336896.2024.2343535 |
| work_keys_str_mv | AT tiagofouteiro prionmeeting2023implicationsofagrowingfield AT tuanecrgvieira prionmeeting2023implicationsofagrowingfield |