Insights into prion strains and neurotoxicity
Top Cited Papers
- 1 July 2007
- journal article
- review article
- Published by Springer Nature in Nature Reviews Molecular Cell Biology
- Vol. 8 (7) , 552-561
- https://doi.org/10.1038/nrm2204
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 PrP(Sc), an aggregated moiety of the host-derived membrane glycolipoprotein PrP(C). Although PrP(C) 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 PrP(Sc) 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.Keywords
This publication has 137 references indexed in Scilit:
- Lethal recessive myelin toxicity of prion protein lacking its central domainThe EMBO Journal, 2007
- Prion infections, blood and transfusionsNature Clinical Practice Neurology, 2006
- The most infectious prion protein particlesNature, 2005
- Prions as adaptive conduits of memory and inheritanceNature Reviews Genetics, 2005
- Probing Copper2+ Binding to the Prion Protein Using Diamagnetic Nickel2+ and 1H NMR: The Unstructured N terminus Facilitates the Coordination of Six Copper2+ Ions at Physiological ConcentrationsJournal of Molecular Biology, 2005
- GFP-tagged prion protein is correctly localized and functionally active in the brains of transgenic miceNeurobiology of Disease, 2004
- Mutant PrP Is Delayed in Its Exit from the Endoplasmic Reticulum, but Neither Wild-type nor Mutant PrP Undergoes Retrotranslocation Prior to Proteasomal DegradationJournal of Biological Chemistry, 2003
- Soluble Dimeric Prion Protein Binds PrPSc In Vivo and Antagonizes Prion DiseaseCell, 2003
- A 'unified theory' of prion propagationNature, 1991
- Temporary and Permanent Modifications to a Single Strain of Mouse Scrapie on Transmission to Rats and HamstersJournal of General Virology, 1987