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Prion Disease and the Innate Immune System
The Roslin Institute and Royal (Dick) School of Veterinary Studies, The University of Edinburgh, Easter Bush Campus, Midlothian, EH25 9RG, UK
* Author to whom correspondence should be addressed.
Received: 6 October 2012; in revised form: 14 November 2012 / Accepted: 22 November 2012 / Published: 28 November 2012
Abstract: Prion diseases or transmissible spongiform encephalopathies are a unique category of infectious protein-misfolding neurodegenerative disorders. Hypothesized to be caused by misfolding of the cellular prion protein these disorders possess an infectious quality that thrives in immune-competent hosts. While much has been discovered about the routing and critical components involved in the peripheral pathogenesis of these agents there are still many aspects to be discovered. Research into this area has been extensive as it represents a major target for therapeutic intervention within this group of diseases. The main focus of pathological damage in these diseases occurs within the central nervous system. Cells of the innate immune system have been proven to be critical players in the initial pathogenesis of prion disease, and may have a role in the pathological progression of disease. Understanding how prions interact with the host innate immune system may provide us with natural pathways and mechanisms to combat these diseases prior to their neuroinvasive stage. We present here a review of the current knowledge regarding the role of the innate immune system in prion pathogenesis.
Keywords: prion disease pathogenesis; transmissible spongiform encephalopathy; innate immune system
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Bradford, B.M.; Mabbott, N.A. Prion Disease and the Innate Immune System. Viruses 2012, 4, 3389-3419.
Bradford BM, Mabbott NA. Prion Disease and the Innate Immune System. Viruses. 2012; 4(12):3389-3419.
Bradford, Barry M.; Mabbott, Neil A. 2012. "Prion Disease and the Innate Immune System." Viruses 4, no. 12: 3389-3419.