prion disease and the innate immune system朊病毒疾病,先天免疫系统.pdfVIP

Viruses 2012, 4, 3389-3419; doi:10.3390/v4123389 OPEN ACCESS viruses ISSN 1999-4915 /journal/viruses Review Prion Disease and the Innate Immune System Barry M. Bradford and Neil A. Mabbott * The Roslin Institute and Royal (Dick) School of Veterinary Studies, The University of Edinburgh, Easter Bush Campus, Midlothian, EH25 9RG, UK; E-Mail: barry.bradford@roslin.ed.ac.uk * Author to whom correspondence should be addressed; E-Mail: neil.mabbott@roslin.ed.ac.uk; Tel.: +44-131-651-9100; Fax: +44-131-651-9105. 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