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Transgenic models of the transmissible spongiform encephalopathies

Published online by Cambridge University Press:  12 February 2004

Jean C. Manson  and
Nadia L. Tuzi
Jean C. Manson
Affiliation:
Institute for Animal Health, Neuropathogenesis Unit, Ogston Building, West Mains Road, Edinburgh, EH9 3JF, UK.
Nadia L. Tuzi
Affiliation:
Institute for Animal Health, Neuropathogenesis Unit, Ogston Building, West Mains Road, Edinburgh, EH9 3JF, UK.
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Abstract

The transmissible spongiform encephalopathies (TSEs) are a group of fatal neurodegenerative diseases that are also transmissible. The PrP protein is central to the disease process and has been hypothesised to be the infectious agent. Polymorphisms in the PrP gene have been linked to the incubation time of TSE disease, and mutations in the human PrP gene have been suggested to result in genetic TSE disease. Several PrP transgenic models have been developed. These models express PrP from different species, with and without PrP mutations, and at different levels of gene expression. This article discusses the contribution of these transgenic models to our present understanding of the TSE diseases.

Keywords

PrPtransgenicgene targetingscrapieCJDCreutzfelt–Jakob diseaseGSSGerstmann–Straussler–Scheinker syndromepriontransmissible spongiform encephalopathiesTSEsneurodegenerative disease
Type
Review Article
Information
Expert Reviews in Molecular Medicine , Volume 3 , Issue 12 , 11 May 2001 , pp. 1 - 15
Copyright
© Cambridge University Press 2001

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