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Review
. 2012 Dec;4(12):3389-419.
doi: 10.3390/v4123389.

Prion disease and the innate immune system

Barry M Bradford  1 , Neil A Mabbott
Affiliations
Review

Prion disease and the innate immune system

Barry M Bradford et al. Viruses. 2012 Dec.

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.

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Figures

Figure 1
Figure 1
Prion disease and the innate immune system. Adapted with permission from motifolio.com, Biomedical PowerPoint Toolkit Suite.
Figure 2
Figure 2
(a) Prnp expression profiling using Affymetrix GeneChip Mouse Genome 430 2.0 Array probesets 1416130_at and (b) 1448233_at; data from [176]. BM, bone marrow; HSC, hematopoietic stem cells; CMP, common myeloid progenitor; GMP, granulocyte-macrophage progenitor; CLP, common lymphoid progenitor; BMDM, bone marrow-derived macrophage; LC, Langerhans cell; BMDC, bone marrow-derived dendritic cell; DC, dendritic cell; pDC, plasmacytoid dendritic cell; IKDC, interferon-producing killer dendritic cell; NK, natural killer.
Figure 3
Figure 3
Wheel network diagram showing the overlap of the major upstream regulators of genes implicated in prion pathogenesis. The network was generated through the use of IPA (Ingenuity® Systems, www.ingenuity.com).
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