dc.contributor.author | Lehmann, S | fr_FR |
dc.date.accessioned | 2012-07-16T10:41:10Z | |
dc.date.available | 2012-07-16T10:41:10Z | |
dc.date.issued | 1996 | fr_FR |
dc.identifier.citation | Lehmann, S, Le rôle de la protéine du prion dans les encéphalopathies spongiformes transmissibles humaines., Med Sci (Paris), 1996, Vol. 12, N° 8-9; p.949-58 | fr_FR |
dc.identifier.issn | 1958-5381 | fr_FR |
dc.identifier.uri | http://hdl.handle.net/10608/852 | |
dc.description.abstract | Les encéphalopathies spongiformes transmissibles forment
un groupe très particulier d’affections neurodégénératives,
d’origine à la fois infectieuse et génétique. L’hypothèse en
faveur actuellement donne à une protéine, appelée la protéine
du prion (PrP), un rôle central dans l’étiologie de ces
affections. Qu’une protéine s’avère être elle-même un agent
infectieux va à l’encontre des notions établies. Des travaux
récents confortent pourtant cette hypothèse et suggèrent que
les mécanismes impliqués dans la pathogénie pourraient dépasser
le cadre des seules encéphalopathies spongiformes. | fr |
dc.description.abstract | The spongiform encephalopathies are a group of fatal neurodegenerative diseases that includes Creutzfeldt-Jakob disease, kuru, Gerstmann-Straussler syndrome, and fatal familial insomnia in human beings. Also called prion diseases, these disorders are unique in being both infectious and genetic in origin. Mutations in the human gene that encodes the prion protein (PrP) are strongly linked to inherited forms of the disease. The agent responsible for infectious forms of the disease is thought to be a protein called PrP(Sc). PrP(Sc) corresponds to an altered form of the normal PrP(C). Several pieces of evidence indicate that PrP(Sc) and PrP(C) do not differ in their primary amino acid sequence, but rather in their conformations. Prion 'replication' is believed to occur when PrP(Sc) in the infecting inoculum interacts specifically with host PrP(C), catalyzing its conversion into PrP(Sc). Recent in vitro studies as well as the use of transgenic animals have given new insights into the comprehension of the mechanisms of these diseases. The conversion of PrP(C) into PrP(Sc) is now considered as the central event in the transmission as well as in the pathogenic process of prion diseases. | en |
dc.language.iso | fr | fr_FR |
dc.publisher | John Libbey Eurotext, Montrouge | fr_FR |
dc.rights | Article en libre accès | fr |
dc.rights | Médecine/Sciences - Inserm - SRMS | fr |
dc.source | M/S. Médecine sciences [revue papier, ISSN : 0767-0974], 1996, Vol. 12, N° 8-9; p.949-58 | fr_FR |
dc.title | Le rôle de la protéine du prion dans les encéphalopathies spongiformes transmissibles humaines. | fr |
dc.title.alternative | Role of the prion protein in human spongiform encephalopathies. | fr_FR |
dc.type | Article | fr_FR |
dc.contributor.affiliation | Washington University, School of Medicine, Box 8228, Dept. cell biology and physiology, 660 South Euclid Avenue,St-Louis, MO 63110; United States. | - |
dc.identifier.doi | 10.4267/10608/852 | |