Creutzfeldt-Jakob disease and mad cows: lessons learnt from yeast cells
| Autor: | Ina Vorberg, Verena Arndt, Hanna Wolf, Andrea Grassmann, James F. Graham, Julia Hofmann |
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| Rok vydání: | 2012 |
| Předmět: |
Prions
animal diseases Disease cytology [Yeasts] Models Biological Creutzfeldt-Jakob Syndrome genetics [Encephalopathy Bovine Spongiform] microbiology [Encephalopathy Bovine Spongiform] Yeasts transmission [Encephalopathy Bovine Spongiform] Animals Humans Medicine ddc:610 Epigenetics metabolism [Yeasts] pathogenicity [Prions] Genetics Transmissible spongiform encephalopathy genetics [Prions] business.industry Neurodegeneration Inheritance (genetic algorithm) genetics [Creutzfeldt-Jakob Syndrome] General Medicine medicine.disease Virology Yeast nervous system diseases Fungal prion Encephalopathy Bovine Spongiform microbiology [Creutzfeldt-Jakob Syndrome] metabolism [Prions] transmission [Creutzfeldt-Jakob Syndrome] Cattle business Biogenesis |
| Zdroj: | Swiss medical weekly 142, w13505 (2012). doi:10.4414/smw.2012.13505 |
| ISSN: | 1424-3997 1424-7860 |
| DOI: | 10.4414/smw.2012.13505 |
| Popis: | Summary Transmissible spongiform encephalopathies are fatal neurodegenerative diseases that affect mammals including humans. The proteinaceous nature of the infectious agent, the prion, and its propagation, challenge established dogmas in biology. It is now widely accepted that prion diseases are caused by unconventional agents principally composed of a misfolded host-encoded protein, PrP. Surprisingly, major break-throughs in prion research came from studies on functionally unrelated proteins in yeast and filamentous fungi. Aggregates composed of these proteins act as epigenetic elements of inheritance that can propagate their alternative states by a conformational switch into an ordered s-sheet rich polymer just like mammalian prions. Since their discovery prions of lower eukaryotes have provided invaluable insights into all aspects of prion biogenesis. Importantly, yeast prions provide proof-of-principle that distinct protein conformers can be infectious and can serve as genetic elements that have the capacity to encipher strain specific information. As a powerful and tractable model system, yeast prions will continue to increase our understanding of prion-host cell interaction and potential mechanisms of protein-based epigenetic inheritance. |
| Databáze: | OpenAIRE |
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