Hypothèse : le modèle du lieu de rencontre pour la maladie des prions
| Autor: | Chantal Monnier, Jean Caston, Dominique Cellier, Gavain M. A. Sweetman, Jean-Marc Valleton, Vic Norris |
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| Rok vydání: | 1997 |
| Předmět: |
Gene isoform
Genetics Ecology animal diseases Bovine spongiform encephalopathy Molecular Structure of Nucleic Acids: A Structure for Deoxyribose Nucleic Acid Scrapie Biology medicine.disease Phenotype General Biochemistry Genetics and Molecular Biology nervous system diseases Chaperonin Biochemistry medicine lipids (amino acids peptides and proteins) Spongiform encephalopathy Gene |
| Zdroj: | Comptes Rendus de l'Académie des Sciences - Series III - Sciences de la Vie. 320:393-398 |
| ISSN: | 0764-4469 |
| DOI: | 10.1016/s0764-4469(97)85027-5 |
| Popis: | Prions are responsible for spongiform diseases such as scrapie and bovine spongiform encephalopathy. It is now generally accepted that the disease mechanism involves the conversion from the normal form, PrPC, to the pathogenic form, PrPSc, and that this isoform is infectious. In the case of scrapie, 15 different forms of the disease have been described and some of these different phenotypes can be conferred by infectious prions that are themselves encoded by normal genes. We propose here that a prion with an altered structure has a correspondingly altered preference for lipids; this altered preference creates a proteolipid domain containing different lipids and other factors such as chaperonins and enzymes responsible for post-translational modifications. Normal prions associated with this abnormal domain adopt the conformation dictated by its lipidic composition (and by the other factors present) and so acquire the lipidic preference of the original pathogenic prions. These transformed prions could then create new proteolipid domains. This process may be considered as semi-conservative replication in which prion and lipids are analogous to the Watson and Crick strands and the proteolipid domain to the double helix itself. |
| Databáze: | OpenAIRE |
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