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Medial Temporal Lobe Involvement in Human Prion Diseases: Implications for the Study of Focal Non Prion Neurodegenerative Pathology
 
 
Review

Neuropathology of Animal Prion Diseases

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Animal and Veterinary Research Centre (CECAV), Associate Laboratory for Animal and Veterinary Science–AL4AnimalS, University of Trás-os-Montes and Alto Douro (UTAD), 5000-801 Vila Real, Portugal
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Pathology Laboratory, UEISPSA, National Institute for Agricultural and Veterinary Research (INIAV), I.P., 2780-157 Oeiras, Portugal
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Pathology Laboratory, UEISPSA, National Institute for Agricultural and Veterinary Research (INIAV), I.P., 4485-655 Vairão-Vila do Conde, Portugal
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Centre for the Research and Technology of Agro-Environmental and Biological Sciences (CITAB), Associate Laboratory Institute for innovation, capacity building and sustainability of agri-food production-Inov4Agro, University of Trás-os-Montes and Alto Douro (UTAD), 5000-801 Vila Real, Portugal
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Research Center for Natural Resources, Environment and Society (CERNAS), Polytechnic Institute of Castelo Branco (IPCB), Av. Pedro Álvares Cabral, 12, 6000-084 Castelo Branco, Portugal
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Quality of Life in the Rural World (Q-Rural), Polytechnic Institute of Castelo Branco (IPCB), Av. Pedro Álvares Cabral, 12, 6000-084 Castelo Branco, Portugal
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Authors to whom correspondence should be addressed.
Academic Editor: Joaquín Castilla
Biomolecules 2021, 11(3), 466; https://doi.org/10.3390/biom11030466
Received: 31 January 2021 / Revised: 4 March 2021 / Accepted: 18 March 2021 / Published: 21 March 2021
(This article belongs to the Special Issue Prion Diseases: A Natural Model for Neurodegenerative Disorders)
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 (PrPC) into a misfolded pathological isoform (PrPSc or prion- proteinaceous infectious particle) that self-propagates by conformational conversion of PrPC. Yet by an unknown mechanism, PrPC can fold into different PrPSc 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 PrPSc 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). View Full-Text
Keywords: prion; neuropathology; spongiform degeneration; animal TSE; neuroinflammation; gene PRNP prion; neuropathology; spongiform degeneration; animal TSE; neuroinflammation; gene PRNP
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MDPI and ACS Style

Orge, L.; Lima, C.; Machado, C.; Tavares, P.; Mendonça, P.; Carvalho, P.; Silva, J.; Pinto, M.d.L.; Bastos, E.; Pereira, J.C.; Gonçalves-Anjo, N.; Gama, A.; Esteves, A.; Alves, A.; Matos, A.C.; Seixas, F.; Silva, F.; Pires, I.; Figueira, L.; Vieira-Pinto, M.; Sargo, R.; Pires, M.d.A. Neuropathology of Animal Prion Diseases. Biomolecules 2021, 11, 466. https://doi.org/10.3390/biom11030466

AMA Style

Orge L, Lima C, Machado C, Tavares P, Mendonça P, Carvalho P, Silva J, Pinto MdL, Bastos E, Pereira JC, Gonçalves-Anjo N, Gama A, Esteves A, Alves A, Matos AC, Seixas F, Silva F, Pires I, Figueira L, Vieira-Pinto M, Sargo R, Pires MdA. Neuropathology of Animal Prion Diseases. Biomolecules. 2021; 11(3):466. https://doi.org/10.3390/biom11030466

Chicago/Turabian Style

Orge, Leonor, Carla Lima, Carla Machado, Paula Tavares, Paula Mendonça, Paulo Carvalho, João Silva, Maria de Lurdes Pinto, Estela Bastos, Jorge Cláudio Pereira, Nuno Gonçalves-Anjo, Adelina Gama, Alexandra Esteves, Anabela Alves, Ana Cristina Matos, Fernanda Seixas, Filipe Silva, Isabel Pires, Luis Figueira, Madalena Vieira-Pinto, Roberto Sargo, and Maria dos Anjos Pires. 2021. "Neuropathology of Animal Prion Diseases" Biomolecules 11, no. 3: 466. https://doi.org/10.3390/biom11030466

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