Quick Search:

uzh logo
Browse by:
bullet
bullet
bullet
bullet

Zurich Open Repository and Archive

Permanent URL to this publication: http://dx.doi.org/10.5167/uzh-1921

Brandner, S; Klein, M A; Frigg, R; Pekarik, V; Parizek, P; Raeber, A; Glatzel, M; Schwarz, P; Rülicke, T; Weissmann, C; Aguzzi, A (2000). Neuroinvasion of prions: insights from mouse models. Experimental Physiology, 85(6):705-712.

[img]
Preview
PDF
1MB

View at publisher

Abstract

The prion was defined by Stanley B. Prusiner as the infectious agent that causes transmissible spongiform encephalopathies. A pathological protein accumulating in the brain of scrapie-infected hamsters was isolated in 1982 and termed prion protein (PrPSc). Its cognate gene Prnp was identified more than a decade ago by Charles Weissmann, and shown to encode the host protein PrP(C). Since the latter discovery, transgenic mice have contributed many important insights into the field of prion biology, including the understanding of the molecular basis of the species barrier for prions. By disrupting the Prnp gene, it was shown that an organism that lacks PrP(C) is resistant to infection by prions. Introduction of mutant PrP genes into PrP-deficient mice was used to investigate the structure-activity relationship of the PrP gene with regard to scrapie susceptibility. Ectopic expression of PrP in PrP knockout mice proved a useful tool for the identification of host cells competent for prion replication. Finally, the availability of PrP knockout mice and transgenic mice overexpressing PrP allows selective reconstitution experiments aimed at expressing PrP in neurografts or in specific populations of haemato- and lymphopoietic cells. The latter studies have allowed us to clarify some of the mechanisms of prion spread and disease pathogenesis.

Citations

Altmetrics

Downloads

28 downloads since deposited on 11 Feb 2008
11 downloads since 12 months

Detailed statistics

Additional indexing

Item Type:Journal Article, refereed
Communities & Collections:04 Faculty of Medicine > University Hospital Zurich > Institute of Neuropathology
DDC:570 Life sciences; biology
610 Medicine & health
Language:English
Date:1 November 2000
Deposited On:11 Feb 2008 12:26
Last Modified:27 Nov 2013 20:03
Publisher:Wiley-Blackwell
ISSN:0958-0670
Publisher DOI:10.1111/j.1469-445X.2000.02091.x
Related URLs:http://ep.physoc.org/cgi/content/abstract/85/6/705
PubMed ID:11187965

Users (please log in): suggest update or correction for this item

Repository Staff Only: item control page