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Aguzzi, A; Blättler, T; Klein, M A; Räber, A J; Hegyi, I; Frigg, R; Brandner, S; Weissmann, C (1997). Tracking prions: the neurografting approach. Cellular and Molecular Life Sciences, 53(6):485-495.

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The physical nature of the agent that causes transmissible spongiform encephalopathies (the 'prion'), is the subject of passionate controversy. Investigation of it has benefited tremendously from the use of transgenic and knockout technologies. However, prion diseases present several other enigmas, including the mechanism of brain damage and how the affinity of the agent for the central nervous system is controlled. Here we show that such questions can be effectively addressed in transgenic and knockout systems, and that pathogenesis may be clarified even before we can be certain about the nature of the infectious agent. Availability of mice overexpressing the Prnp gene (which encodes the normal prion protein) and Prnp knockout mice allows for selective reconstitution experiments aimed at expressing PrP in specific portions of the brain or in selected populations of hemato- and lymphopoietic origin. We summarize how such studies can offer insights into how prions administered to peripheral sites can gain access to central nervous tissue, and into the molecular requirements for spongiform brain damage.


7 citations in Web of Science®
8 citations in Scopus®
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Additional indexing

Item Type:Journal Article, refereed
Communities & Collections:04 Faculty of Medicine > University Hospital Zurich > Institute of Neuropathology
Dewey Decimal Classification:570 Life sciences; biology
610 Medicine & health
Date:1 June 1997
Deposited On:11 Feb 2008 12:25
Last Modified:05 Apr 2016 12:20
Publisher DOI:10.1007/s000180050060
PubMed ID:9230924

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