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Neuropathology of Animal Prion Diseases
Transmissible Spongiform Encephalopathies (TSEs) or prion diseases are a fatal group of infectious, inherited and spontaneous neurodegenerative diseases affecting human and animals. They are caused by the conversion of cellular prion protein (PrP(C)) into a misfolded pathological isoform (PrP(Sc) or...
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/PMC8004141/ https://www.ncbi.nlm.nih.gov/pubmed/33801117 http://dx.doi.org/10.3390/biom11030466 |
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author | Orge, Leonor Lima, Carla Machado, Carla Tavares, Paula Mendonça, Paula Carvalho, Paulo Silva, João Pinto, Maria de Lurdes Bastos, Estela Pereira, Jorge Cláudio Gonçalves-Anjo, Nuno Gama, Adelina Esteves, Alexandra Alves, Anabela Matos, Ana Cristina Seixas, Fernanda Silva, Filipe Pires, Isabel Figueira, Luis Vieira-Pinto, Madalena Sargo, Roberto Pires, Maria dos Anjos |
author_facet | Orge, Leonor Lima, Carla Machado, Carla Tavares, Paula Mendonça, Paula Carvalho, Paulo Silva, João Pinto, Maria de Lurdes Bastos, Estela Pereira, Jorge Cláudio Gonçalves-Anjo, Nuno Gama, Adelina Esteves, Alexandra Alves, Anabela Matos, Ana Cristina Seixas, Fernanda Silva, Filipe Pires, Isabel Figueira, Luis Vieira-Pinto, Madalena Sargo, Roberto Pires, Maria dos Anjos |
author_sort | Orge, Leonor |
collection | PubMed |
description | Transmissible Spongiform Encephalopathies (TSEs) or prion diseases are a fatal group of infectious, inherited and spontaneous neurodegenerative diseases affecting human and animals. They are caused by the conversion of cellular prion protein (PrP(C)) into a misfolded pathological isoform (PrP(Sc) or prion- proteinaceous infectious particle) that self-propagates by conformational conversion of PrP(C). Yet by an unknown mechanism, PrP(C) can fold into different PrP(Sc) conformers that may result in different prion strains that display specific disease phenotype (incubation time, clinical signs and lesion profile). Although the pathways for neurodegeneration as well as the involvement of brain inflammation in these diseases are not well understood, the spongiform changes, neuronal loss, gliosis and accumulation of PrP(Sc) are the characteristic neuropathological lesions. Scrapie affecting small ruminants was the first identified TSE and has been considered the archetype of prion diseases, though atypical and new animal prion diseases continue to emerge highlighting the importance to investigate the lesion profile in naturally affected animals. In this report, we review the neuropathology and the neuroinflammation of animal prion diseases in natural hosts from scrapie, going through the zoonotic bovine spongiform encephalopathy (BSE), the chronic wasting disease (CWD) to the newly identified camel prion disease (CPD). |
format | Online Article Text |
id | pubmed-8004141 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2021 |
publisher | MDPI |
record_format | MEDLINE/PubMed |
spelling | pubmed-80041412021-03-28 Neuropathology of Animal Prion Diseases Orge, Leonor Lima, Carla Machado, Carla Tavares, Paula Mendonça, Paula Carvalho, Paulo Silva, João Pinto, Maria de Lurdes Bastos, Estela Pereira, Jorge Cláudio Gonçalves-Anjo, Nuno Gama, Adelina Esteves, Alexandra Alves, Anabela Matos, Ana Cristina Seixas, Fernanda Silva, Filipe Pires, Isabel Figueira, Luis Vieira-Pinto, Madalena Sargo, Roberto Pires, Maria dos Anjos Biomolecules Review Transmissible Spongiform Encephalopathies (TSEs) or prion diseases are a fatal group of infectious, inherited and spontaneous neurodegenerative diseases affecting human and animals. They are caused by the conversion of cellular prion protein (PrP(C)) into a misfolded pathological isoform (PrP(Sc) or prion- proteinaceous infectious particle) that self-propagates by conformational conversion of PrP(C). Yet by an unknown mechanism, PrP(C) can fold into different PrP(Sc) conformers that may result in different prion strains that display specific disease phenotype (incubation time, clinical signs and lesion profile). Although the pathways for neurodegeneration as well as the involvement of brain inflammation in these diseases are not well understood, the spongiform changes, neuronal loss, gliosis and accumulation of PrP(Sc) are the characteristic neuropathological lesions. Scrapie affecting small ruminants was the first identified TSE and has been considered the archetype of prion diseases, though atypical and new animal prion diseases continue to emerge highlighting the importance to investigate the lesion profile in naturally affected animals. In this report, we review the neuropathology and the neuroinflammation of animal prion diseases in natural hosts from scrapie, going through the zoonotic bovine spongiform encephalopathy (BSE), the chronic wasting disease (CWD) to the newly identified camel prion disease (CPD). MDPI 2021-03-21 /pmc/articles/PMC8004141/ /pubmed/33801117 http://dx.doi.org/10.3390/biom11030466 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 (http://creativecommons.org/licenses/by/4.0/ (https://creativecommons.org/licenses/by/4.0/) ). |
spellingShingle | Review Orge, Leonor Lima, Carla Machado, Carla Tavares, Paula Mendonça, Paula Carvalho, Paulo Silva, João Pinto, Maria de Lurdes Bastos, Estela Pereira, Jorge Cláudio Gonçalves-Anjo, Nuno Gama, Adelina Esteves, Alexandra Alves, Anabela Matos, Ana Cristina Seixas, Fernanda Silva, Filipe Pires, Isabel Figueira, Luis Vieira-Pinto, Madalena Sargo, Roberto Pires, Maria dos Anjos Neuropathology of Animal Prion Diseases |
title | Neuropathology of Animal Prion Diseases |
title_full | Neuropathology of Animal Prion Diseases |
title_fullStr | Neuropathology of Animal Prion Diseases |
title_full_unstemmed | Neuropathology of Animal Prion Diseases |
title_short | Neuropathology of Animal Prion Diseases |
title_sort | neuropathology of animal prion diseases |
topic | Review |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8004141/ https://www.ncbi.nlm.nih.gov/pubmed/33801117 http://dx.doi.org/10.3390/biom11030466 |
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