Transgenesis Applied to Transmissible Spongiform Encephalopathies View Full Text


Ontology type: schema:ScholarlyArticle     


Article Info

DATE

2002-12

AUTHORS

Jean-Luc Vilotte, Hubert Laude

ABSTRACT

Transmissible spongiform encephalopathies (TSE) are fatal neurodegenerative disorders present in various mammals. TSEs have been studies intensively, even more so following the BSE crisis and the subsequent threat of a human nvCJD epidemic. In the 'protein-only' hypothesis, the infectious agent, called prion, is assumed to be a misfolded host protein. Transgenesis has mainly been applied to study the role of this protein, its structure-function relationship with respect to its pathogenic properties and to assess the genetic origin of the well-recognised species barrier effect. This approach has somewhat supplemented the lack of in vitro models. This review will try to summarise the impressive work that has been done in this field. Although many questions remain unanswered, transgenic experiments have and will still improve our knowledge on this disease and might help us to develop critically needed therapeutic approaches. More... »

PAGES

547-564

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  • Identifiers

    URI

    http://scigraph.springernature.com/pub.10.1023/a:1021125510220

    DOI

    http://dx.doi.org/10.1023/a:1021125510220

    DIMENSIONS

    https://app.dimensions.ai/details/publication/pub.1006871118

    PUBMED

    https://www.ncbi.nlm.nih.gov/pubmed/12509129


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