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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...

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Autores principales: Toledano-Díaz, Adolfo, Álvarez, María Isabel, Rodríguez, Jose-Julio, Badiola, Juan Jose, Monzón, Marta, Toledano, Adolfo
Formato: Online Artículo Texto
Lenguaje:English
Publicado: MDPI 2021
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.
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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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