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Reflections on Cerebellar Neuropathology in Classical Scrapie
In this review, the most important neuropathological changes found in the cerebella of sheep affected by classical natural scrapie are discussed. This disease is the oldest known of a group of unconventional “infections” caused by toxic prions of different origins. Scrapie is currently considered a...
Autores principales: | , , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
MDPI
2021
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8146067/ https://www.ncbi.nlm.nih.gov/pubmed/33924986 http://dx.doi.org/10.3390/biom11050649 |
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author | Toledano-Díaz, Adolfo Álvarez, María Isabel Rodríguez, Jose-Julio Badiola, Juan Jose Monzón, Marta Toledano, Adolfo |
author_facet | Toledano-Díaz, Adolfo Álvarez, María Isabel Rodríguez, Jose-Julio Badiola, Juan Jose Monzón, Marta Toledano, Adolfo |
author_sort | Toledano-Díaz, Adolfo |
collection | PubMed |
description | In this review, the most important neuropathological changes found in the cerebella of sheep affected by classical natural scrapie are discussed. This disease is the oldest known of a group of unconventional “infections” caused by toxic prions of different origins. Scrapie is currently considered a “transmissible spongiform encephalopathy” (due to its neuropathological characteristics and its transmission), which is the paradigm of prion pathologies as well as many encephalopathies (prion-like) that present aberrant deposits of insoluble protein with neurotoxic effects due to errors in their catabolization (“misfolding protein diseases”). The study of this disease is, therefore, of great relevance. Our work data from the authors’ previous publications as well as other research in the field. The four most important types of neuropathological changes are neuron abnormalities and loss, neurogliosis, tissue vacuolization (spongiosis) and pathological or abnormal prion protein (PrP) deposits/deposition. These findings were analyzed and compared to other neuropathologies. Various aspects related to the presentation and progression of the disease, the involution of different neuronal types, the neuroglial responses and the appearance of abnormal PrP deposits are discussed. The most important points of controversy in scrapie neuropathology are presented. |
format | Online Article Text |
id | pubmed-8146067 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2021 |
publisher | MDPI |
record_format | MEDLINE/PubMed |
spelling | pubmed-81460672021-05-26 Reflections on Cerebellar Neuropathology in Classical Scrapie Toledano-Díaz, Adolfo Álvarez, María Isabel Rodríguez, Jose-Julio Badiola, Juan Jose Monzón, Marta Toledano, Adolfo Biomolecules Review In this review, the most important neuropathological changes found in the cerebella of sheep affected by classical natural scrapie are discussed. This disease is the oldest known of a group of unconventional “infections” caused by toxic prions of different origins. Scrapie is currently considered a “transmissible spongiform encephalopathy” (due to its neuropathological characteristics and its transmission), which is the paradigm of prion pathologies as well as many encephalopathies (prion-like) that present aberrant deposits of insoluble protein with neurotoxic effects due to errors in their catabolization (“misfolding protein diseases”). The study of this disease is, therefore, of great relevance. Our work data from the authors’ previous publications as well as other research in the field. The four most important types of neuropathological changes are neuron abnormalities and loss, neurogliosis, tissue vacuolization (spongiosis) and pathological or abnormal prion protein (PrP) deposits/deposition. These findings were analyzed and compared to other neuropathologies. Various aspects related to the presentation and progression of the disease, the involution of different neuronal types, the neuroglial responses and the appearance of abnormal PrP deposits are discussed. The most important points of controversy in scrapie neuropathology are presented. MDPI 2021-04-28 /pmc/articles/PMC8146067/ /pubmed/33924986 http://dx.doi.org/10.3390/biom11050649 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 | Review Toledano-Díaz, Adolfo Álvarez, María Isabel Rodríguez, Jose-Julio Badiola, Juan Jose Monzón, Marta Toledano, Adolfo Reflections on Cerebellar Neuropathology in Classical Scrapie |
title | Reflections on Cerebellar Neuropathology in Classical Scrapie |
title_full | Reflections on Cerebellar Neuropathology in Classical Scrapie |
title_fullStr | Reflections on Cerebellar Neuropathology in Classical Scrapie |
title_full_unstemmed | Reflections on Cerebellar Neuropathology in Classical Scrapie |
title_short | Reflections on Cerebellar Neuropathology in Classical Scrapie |
title_sort | reflections on cerebellar neuropathology in classical scrapie |
topic | Review |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8146067/ https://www.ncbi.nlm.nih.gov/pubmed/33924986 http://dx.doi.org/10.3390/biom11050649 |
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