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Review

The Effects of Immune System Modulation on Prion Disease Susceptibility and Pathogenesis

The Roslin Institute & Royal (Dick) School of Veterinary Studies, University of Edinburgh, Easter Bush, Midlothian EH25 9RG, UK
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Int. J. Mol. Sci. 2020, 21(19), 7299; https://doi.org/10.3390/ijms21197299
Received: 9 September 2020 / Revised: 25 September 2020 / Accepted: 29 September 2020 / Published: 2 October 2020
(This article belongs to the Special Issue Prions and Prion Diseases)
Prion diseases are a unique group of infectious chronic neurodegenerative disorders to which there are no cures. Although prion infections do not stimulate adaptive immune responses in infected individuals, the actions of certain immune cell populations can have a significant impact on disease pathogenesis. After infection, the targeting of peripherally-acquired prions to specific immune cells in the secondary lymphoid organs (SLO), such as the lymph nodes and spleen, is essential for the efficient transmission of disease to the brain. Once the prions reach the brain, interactions with other immune cell populations can provide either host protection or accelerate the neurodegeneration. In this review, we provide a detailed account of how factors such as inflammation, ageing and pathogen co-infection can affect prion disease pathogenesis and susceptibility. For example, we discuss how changes to the abundance, function and activation status of specific immune cell populations can affect the transmission of prion diseases by peripheral routes. We also describe how the effects of systemic inflammation on certain glial cell subsets in the brains of infected individuals can accelerate the neurodegeneration. A detailed understanding of the factors that affect prion disease transmission and pathogenesis is essential for the development of novel intervention strategies. View Full-Text
Keywords: prions and prion disease; immune system; inflammation; aging; co-infection; susceptibility prions and prion disease; immune system; inflammation; aging; co-infection; susceptibility
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MDPI and ACS Style

Mabbott, N.A.; Bradford, B.M.; Pal, R.; Young, R.; Donaldson, D.S. The Effects of Immune System Modulation on Prion Disease Susceptibility and Pathogenesis. Int. J. Mol. Sci. 2020, 21, 7299. https://doi.org/10.3390/ijms21197299

AMA Style

Mabbott NA, Bradford BM, Pal R, Young R, Donaldson DS. The Effects of Immune System Modulation on Prion Disease Susceptibility and Pathogenesis. International Journal of Molecular Sciences. 2020; 21(19):7299. https://doi.org/10.3390/ijms21197299

Chicago/Turabian Style

Mabbott, Neil A., Barry M. Bradford, Reiss Pal, Rachel Young, and David S. Donaldson. 2020. "The Effects of Immune System Modulation on Prion Disease Susceptibility and Pathogenesis" International Journal of Molecular Sciences 21, no. 19: 7299. https://doi.org/10.3390/ijms21197299

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