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[Prion diseases].
Rev Neurol (Paris). 2002 Oct; 158(10 Pt 1):998-1007.RN

Abstract

Creutzfeldt-Jakob disease, kuru, Gerstmann Sträussler Scheinker syndrome and fatal familial insomnia in humans, as well as scrapie and bovine spongiform encephalopathy, in animals, are fatal disorders of the central nervous system that are part of the group of transmissible spongiform encephalopathies, (TSE) or prion diseases. Neuronal intracellular spongiosis and the accumulation of abnormal, protease resistant prion protein in the nervous central system characterize TSE. The conformational change of a host protein, prion protein, into a pathological isoform is the key pathogenetic event in TSE. Despite their relative rarity, prion diseases have a great impact on the scientific community and society in general. There are two major reasons: first, the heretical hypothesis of a disease transmitted by an "infectious protein" in the absence of nucleic acid, the basis of the conformational transmissibility concept; second, the panic originated from the appearance of new variant Creutzfeldt-Jakob disease and the evidence linking it to the exposure of humans to bovine spongiform encephalopathy via food contaminated by affected bovine tissue. Novel therapeutic approaches are examined.

Authors+Show Affiliations

Warter JM
Service de Neurologie, Hôpitaux Universitaires, Strasbourg, France.
Steinmetz G
No affiliation info available
Mohr M
No affiliation info available
Tranchant C
No affiliation info available

MeSH

BrainHumansPrPC ProteinsPrion Diseases

Pub Type(s)

English Abstract
Journal Article
Review

Language

fre

PubMed ID

12407310

Citation

Warter, J M., et al. "[Prion Diseases]." Revue Neurologique, vol. 158, no. 10 Pt 1, 2002, pp. 998-1007.
Warter JM, Steinmetz G, Mohr M, et al. [Prion diseases]. Rev Neurol (Paris). 2002;158(10 Pt 1):998-1007.
Warter, J. M., Steinmetz, G., Mohr, M., & Tranchant, C. (2002). [Prion diseases]. Revue Neurologique, 158(10 Pt 1), 998-1007.
Warter JM, et al. [Prion Diseases]. Rev Neurol (Paris). 2002;158(10 Pt 1):998-1007. PubMed PMID: 12407310.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - [Prion diseases]. AU - Warter,J M, AU - Steinmetz,G, AU - Mohr,M, AU - Tranchant,C, PY - 2002/10/31/pubmed PY - 2003/1/7/medline PY - 2002/10/31/entrez SP - 998 EP - 1007 JF - Revue neurologique JO - Rev Neurol (Paris) VL - 158 IS - 10 Pt 1 N2 - Creutzfeldt-Jakob disease, kuru, Gerstmann Sträussler Scheinker syndrome and fatal familial insomnia in humans, as well as scrapie and bovine spongiform encephalopathy, in animals, are fatal disorders of the central nervous system that are part of the group of transmissible spongiform encephalopathies, (TSE) or prion diseases. Neuronal intracellular spongiosis and the accumulation of abnormal, protease resistant prion protein in the nervous central system characterize TSE. The conformational change of a host protein, prion protein, into a pathological isoform is the key pathogenetic event in TSE. Despite their relative rarity, prion diseases have a great impact on the scientific community and society in general. There are two major reasons: first, the heretical hypothesis of a disease transmitted by an "infectious protein" in the absence of nucleic acid, the basis of the conformational transmissibility concept; second, the panic originated from the appearance of new variant Creutzfeldt-Jakob disease and the evidence linking it to the exposure of humans to bovine spongiform encephalopathy via food contaminated by affected bovine tissue. Novel therapeutic approaches are examined. SN - 0035-3787 UR - https://www.unboundmedicine.com/medline/citation/12407310/[Prion_diseases]_ DB - PRIME DP - Unbound Medicine ER -