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The Effects of Immune System Modulation on Prion Disease Susceptibility and Pathogenesis
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 patho...
Autores principales: | , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
MDPI
2020
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7582561/ https://www.ncbi.nlm.nih.gov/pubmed/33023255 http://dx.doi.org/10.3390/ijms21197299 |
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author | Mabbott, Neil A. Bradford, Barry M. Pal, Reiss Young, Rachel Donaldson, David S. |
author_facet | Mabbott, Neil A. Bradford, Barry M. Pal, Reiss Young, Rachel Donaldson, David S. |
author_sort | Mabbott, Neil A. |
collection | PubMed |
description | 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. |
format | Online Article Text |
id | pubmed-7582561 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2020 |
publisher | MDPI |
record_format | MEDLINE/PubMed |
spelling | pubmed-75825612020-10-29 The Effects of Immune System Modulation on Prion Disease Susceptibility and Pathogenesis Mabbott, Neil A. Bradford, Barry M. Pal, Reiss Young, Rachel Donaldson, David S. Int J Mol Sci Review 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. MDPI 2020-10-02 /pmc/articles/PMC7582561/ /pubmed/33023255 http://dx.doi.org/10.3390/ijms21197299 Text en © 2020 by the authors. Licensee MDPI, Basel, Switzerland. This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution (CC BY) license (http://creativecommons.org/licenses/by/4.0/). |
spellingShingle | Review Mabbott, Neil A. Bradford, Barry M. Pal, Reiss Young, Rachel Donaldson, David S. The Effects of Immune System Modulation on Prion Disease Susceptibility and Pathogenesis |
title | The Effects of Immune System Modulation on Prion Disease Susceptibility and Pathogenesis |
title_full | The Effects of Immune System Modulation on Prion Disease Susceptibility and Pathogenesis |
title_fullStr | The Effects of Immune System Modulation on Prion Disease Susceptibility and Pathogenesis |
title_full_unstemmed | The Effects of Immune System Modulation on Prion Disease Susceptibility and Pathogenesis |
title_short | The Effects of Immune System Modulation on Prion Disease Susceptibility and Pathogenesis |
title_sort | effects of immune system modulation on prion disease susceptibility and pathogenesis |
topic | Review |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7582561/ https://www.ncbi.nlm.nih.gov/pubmed/33023255 http://dx.doi.org/10.3390/ijms21197299 |
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