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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doaj-d4a8a633a4ea433581f67d490e1866022021-05-31T23:56:47ZengMDPI AGPathogens2076-08172021-05-011059659610.3390/pathogens10050596Novel Morphological Glial Alterations in the Spectrum of Prion Disease Types: A Focus on Common FindingsMoisés Garcés0Isabel M. Guijarro1Diane L. Ritchie2Juan J. Badiola3Marta Monzón4Research Centre for Encephalopathies and Transmissible Emerging Diseases, Institute for Health Research Aragón (IIS), University of Zaragoza, 50013 Zaragoza, SpainResearch Centre for Encephalopathies and Transmissible Emerging Diseases, Institute for Health Research Aragón (IIS), University of Zaragoza, 50013 Zaragoza, SpainNational CJD Research & Surveillance Unit, Centre for Clinical Brain Sciences, The University of Edinburgh, Edinburgh EH4 2XU, UKResearch Centre for Encephalopathies and Transmissible Emerging Diseases, Institute for Health Research Aragón (IIS), University of Zaragoza, 50013 Zaragoza, SpainResearch Centre for Encephalopathies and Transmissible Emerging Diseases, Institute for Health Research Aragón (IIS), University of Zaragoza, 50013 Zaragoza, SpainHuman 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.https://www.mdpi.com/2076-0817/10/5/596astrogliamicrogliainterlaminar astrogliaprion typescortexcerebellum |
collection |
DOAJ |
language |
English |
format |
Article |
sources |
DOAJ |
author |
Moisés Garcés Isabel M. Guijarro Diane L. Ritchie Juan J. Badiola Marta Monzón |
spellingShingle |
Moisés Garcés Isabel M. Guijarro Diane L. Ritchie Juan J. Badiola Marta Monzón Novel Morphological Glial Alterations in the Spectrum of Prion Disease Types: A Focus on Common Findings Pathogens astroglia microglia interlaminar astroglia prion types cortex cerebellum |
author_facet |
Moisés Garcés Isabel M. Guijarro Diane L. Ritchie Juan J. Badiola Marta Monzón |
author_sort |
Moisés Garcés |
title |
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_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_sort |
novel morphological glial alterations in the spectrum of prion disease types: a focus on common findings |
publisher |
MDPI AG |
series |
Pathogens |
issn |
2076-0817 |
publishDate |
2021-05-01 |
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. |
topic |
astroglia microglia interlaminar astroglia prion types cortex cerebellum |
url |
https://www.mdpi.com/2076-0817/10/5/596 |
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