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Novel Morphological Glial Alterations in the Spectrum of Prion Disease Types: A Focus on Common Findings

Human prion diseases are a group of rare fatal neurodegenerative diseases with sporadic, genetic, and acquired forms. They are neuropathologically characterized by pathological prion protein accumulation, neuronal death, and vacuolation. Classical immunological response has long been known not to pl...

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Autores principales: Garcés, Moisés, Guijarro, Isabel M., Ritchie, Diane L., Badiola, Juan J., Monzón, Marta
Formato: Online Artículo Texto
Lenguaje:English
Publicado: MDPI 2021
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8153175/
https://www.ncbi.nlm.nih.gov/pubmed/34068251
http://dx.doi.org/10.3390/pathogens10050596
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author Garcés, Moisés
Guijarro, Isabel M.
Ritchie, Diane L.
Badiola, Juan J.
Monzón, Marta
author_facet Garcés, Moisés
Guijarro, Isabel M.
Ritchie, Diane L.
Badiola, Juan J.
Monzón, Marta
author_sort Garcés, Moisés
collection PubMed
description Human prion diseases are a group of rare fatal neurodegenerative diseases with sporadic, genetic, and acquired forms. They are neuropathologically characterized by pathological prion protein accumulation, neuronal death, and vacuolation. Classical immunological response has long been known not to play a major in prion diseases; however, gliosis is known to be a common feature although variable in extent and poorly described. In this investigation, astrogliosis and activated microglia in two brain regions were assessed and compared with non-neurologically affected patients in a representative sample across the spectrum of Creutzfeldt–Jakob disease (CJD) forms and subtypes in order to analyze the influence of prion strain on pathological processes. In this report, we choose to focus on features common to all CJD types rather than the diversity among them. Novel pathological changes in both glial cell types were found to be shared by all CJD types. Microglial activation correlated to astrogliosis. Spongiosis, but not pathological prion protein deposition, correlated to both astrogliosis and microgliosis. At the ultrastructural level, astrocytic glial filaments correlated with pathological changes associated with prion disease. These observations confirm that neuroglia play a prominent role in the neurodegenerative process of prion diseases, regardless of the causative prion type.
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spelling pubmed-81531752021-05-27 Novel Morphological Glial Alterations in the Spectrum of Prion Disease Types: A Focus on Common Findings Garcés, Moisés Guijarro, Isabel M. Ritchie, Diane L. Badiola, Juan J. Monzón, Marta Pathogens Article Human prion diseases are a group of rare fatal neurodegenerative diseases with sporadic, genetic, and acquired forms. They are neuropathologically characterized by pathological prion protein accumulation, neuronal death, and vacuolation. Classical immunological response has long been known not to play a major in prion diseases; however, gliosis is known to be a common feature although variable in extent and poorly described. In this investigation, astrogliosis and activated microglia in two brain regions were assessed and compared with non-neurologically affected patients in a representative sample across the spectrum of Creutzfeldt–Jakob disease (CJD) forms and subtypes in order to analyze the influence of prion strain on pathological processes. In this report, we choose to focus on features common to all CJD types rather than the diversity among them. Novel pathological changes in both glial cell types were found to be shared by all CJD types. Microglial activation correlated to astrogliosis. Spongiosis, but not pathological prion protein deposition, correlated to both astrogliosis and microgliosis. At the ultrastructural level, astrocytic glial filaments correlated with pathological changes associated with prion disease. These observations confirm that neuroglia play a prominent role in the neurodegenerative process of prion diseases, regardless of the causative prion type. MDPI 2021-05-13 /pmc/articles/PMC8153175/ /pubmed/34068251 http://dx.doi.org/10.3390/pathogens10050596 Text en © 2021 by the authors. https://creativecommons.org/licenses/by/4.0/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 (https://creativecommons.org/licenses/by/4.0/).
spellingShingle Article
Garcés, Moisés
Guijarro, Isabel M.
Ritchie, Diane L.
Badiola, Juan J.
Monzón, Marta
Novel Morphological Glial Alterations in the Spectrum of Prion Disease Types: A Focus on Common Findings
title Novel Morphological Glial Alterations in the Spectrum of Prion Disease Types: A Focus on Common Findings
title_full Novel Morphological Glial Alterations in the Spectrum of Prion Disease Types: A Focus on Common Findings
title_fullStr Novel Morphological Glial Alterations in the Spectrum of Prion Disease Types: A Focus on Common Findings
title_full_unstemmed Novel Morphological Glial Alterations in the Spectrum of Prion Disease Types: A Focus on Common Findings
title_short Novel Morphological Glial Alterations in the Spectrum of Prion Disease Types: A Focus on Common Findings
title_sort novel morphological glial alterations in the spectrum of prion disease types: a focus on common findings
topic Article
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8153175/
https://www.ncbi.nlm.nih.gov/pubmed/34068251
http://dx.doi.org/10.3390/pathogens10050596
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