Review
Nature Reviews Molecular Cell Biology 8, 552-561 (July 2007) | doi:10.1038/nrm2204
Article series: Mechanisms of disease
Insights into prion strains and neurotoxicity
Adriano Aguzzi1, Mathias Heikenwalder1 & Magdalini Polymenidou1 About the authors
Abstract
Transmissible spongiform encephalopathies (TSEs) are neurodegenerative diseases that are caused by prions and affect humans and many animal species. It is now widely accepted that the infectious agent that causes TSEs is PrPSc, an aggregated moiety of the host-derived membrane glycolipoprotein PrPC. Although PrPC is encoded by the host genome, prions themselves encipher many phenotypic TSE variants, known as prion strains. Prion strains are TSE isolates that, after inoculation into distinct hosts, cause disease with consistent characteristics, such as incubation period, distinct patterns of PrPSc distribution and spongiosis and relative severity of the spongiform changes in the brain. The existence of such strains poses a fascinating challenge to prion research.
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Author affiliations
- Institute of Neuropathology, University Hospital of Zürich, Schmelzbergstrasse 12, CH-8091 Zürich, Switzerland.
Correspondence to: Adriano Aguzzi1 Email: adriano.aguzzi@usz.ch
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