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Cell culture models for prion infection Noriyuki Nishida 1 1Department of Molecular Microbiology and Immunology, Nagasaki University, Graduate School of Bio-medical Sciences Keyword: CJD , BSE , プリオン蛋白 , プリオン感染細胞 pp.45-53
Published Date 2003/2/10
DOI https://doi.org/10.11477/mf.1431100282
  • Abstract
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 Transmissible spongiform encephalopathies(TSEs), or prion diseases, are neurodegenerative disorders that include Creutzfeldt-Jakob disease(CJD)and Gerstmann-Strässler syndrome(GSS)in man, and scrapie and bovine spongiform encephalopathy(BSE)in animals. Although the nature of the infectious agent, termed prion, is not fully understood, the posttranslational conversion of the normal cellular prion protein, PrPC, to an abnormal protease-resistant isoform, PrPres, is a key event in the pathogenesis of TSEs and the infectious agents are thought to be composed mainly of this abnormal protein. There still remains the possibility of there existing unidentified molecules in aggregated PrPres, because attempts to reproduce infectious PrP moleculesin vitrohave been unsuccessful. Cell culture models are powerful tools for investigating the nature of the infectious agent and the mechanism of its replication. Experiments using cell cultures susceptible to prion have suggested that successful infection relies upon an adequate expression level of PrPCin the host cell. However, most attempts usingin vitrotransmission have failed and only few cell lines are known to bo susceptible to prion, indicating that unknown host factors may also play an important role in prion infection. Recent data indicate that such cellular factors are not related to the neuronal cell. Further analysis of infectious agents isolated in the pure-cell culture system will elucidate the nature of TSE agents, enabling us to develop new diagnostic tests or tools for prevention of prion infection.


Copyright © 2003, Igaku-Shoin Ltd. All rights reserved.

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電子版ISSN 1882-1243 印刷版ISSN 0001-8724 医学書院

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