{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,2,28]],"date-time":"2026-02-28T00:47:29Z","timestamp":1772239649160,"version":"3.50.1"},"reference-count":178,"publisher":"MDPI AG","issue":"3","license":[{"start":{"date-parts":[[2021,3,21]],"date-time":"2021-03-21T00:00:00Z","timestamp":1616284800000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"name":"Funda\u00e7\u00e3o para a Ci\u00eancia e Tecnologia - FCT-FEDER- Balc\u00e3o2020.","award":["POCI-01-0145-FEDER-029947"],"award-info":[{"award-number":["POCI-01-0145-FEDER-029947"]}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Biomolecules"],"abstract":"<jats:p>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).<\/jats:p>","DOI":"10.3390\/biom11030466","type":"journal-article","created":{"date-parts":[[2021,3,21]],"date-time":"2021-03-21T22:00:37Z","timestamp":1616364037000},"page":"466","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":43,"title":["Neuropathology of Animal Prion Diseases"],"prefix":"10.3390","volume":"11","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-2673-2758","authenticated-orcid":false,"given":"Leonor","family":"Orge","sequence":"first","affiliation":[{"name":"Animal and Veterinary Research Centre (CECAV), Associate Laboratory for Animal and Veterinary Science\u2013AL4AnimalS, University of Tr\u00e1s-os-Montes and Alto Douro (UTAD), 5000-801 Vila Real, Portugal"},{"name":"Pathology Laboratory, UEISPSA, National Institute for Agricultural and Veterinary Research (INIAV), I.P., 2780-157 Oeiras, Portugal"}]},{"given":"Carla","family":"Lima","sequence":"additional","affiliation":[{"name":"Pathology Laboratory, UEISPSA, National Institute for Agricultural and Veterinary Research (INIAV), I.P., 4485-655 Vair\u00e3o-Vila do Conde, Portugal"}]},{"given":"Carla","family":"Machado","sequence":"additional","affiliation":[{"name":"Pathology Laboratory, UEISPSA, National Institute for Agricultural and Veterinary Research (INIAV), I.P., 2780-157 Oeiras, Portugal"}]},{"given":"Paula","family":"Tavares","sequence":"additional","affiliation":[{"name":"Pathology Laboratory, UEISPSA, National Institute for Agricultural and Veterinary Research (INIAV), I.P., 4485-655 Vair\u00e3o-Vila do Conde, Portugal"}]},{"given":"Paula","family":"Mendon\u00e7a","sequence":"additional","affiliation":[{"name":"Pathology Laboratory, UEISPSA, National Institute for Agricultural and Veterinary Research (INIAV), I.P., 2780-157 Oeiras, Portugal"}]},{"given":"Paulo","family":"Carvalho","sequence":"additional","affiliation":[{"name":"Pathology Laboratory, UEISPSA, National Institute for Agricultural and Veterinary Research (INIAV), I.P., 2780-157 Oeiras, Portugal"}]},{"given":"Jo\u00e3o","family":"Silva","sequence":"additional","affiliation":[{"name":"Pathology Laboratory, UEISPSA, National Institute for Agricultural and Veterinary Research (INIAV), I.P., 2780-157 Oeiras, Portugal"}]},{"given":"Maria de Lurdes","family":"Pinto","sequence":"additional","affiliation":[{"name":"Animal and Veterinary Research Centre (CECAV), Associate Laboratory for Animal and Veterinary Science\u2013AL4AnimalS, University of Tr\u00e1s-os-Montes and Alto Douro (UTAD), 5000-801 Vila Real, Portugal"}]},{"given":"Estela","family":"Bastos","sequence":"additional","affiliation":[{"name":"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 Tr\u00e1s-os-Montes and Alto Douro (UTAD), 5000-801 Vila Real, Portugal"}]},{"given":"Jorge Cl\u00e1udio","family":"Pereira","sequence":"additional","affiliation":[{"name":"Animal and Veterinary Research Centre (CECAV), Associate Laboratory for Animal and Veterinary Science\u2013AL4AnimalS, University of Tr\u00e1s-os-Montes and Alto Douro (UTAD), 5000-801 Vila Real, Portugal"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-0586-2550","authenticated-orcid":false,"given":"Nuno","family":"Gon\u00e7alves-Anjo","sequence":"additional","affiliation":[{"name":"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 Tr\u00e1s-os-Montes and Alto Douro (UTAD), 5000-801 Vila Real, Portugal"}]},{"given":"Adelina","family":"Gama","sequence":"additional","affiliation":[{"name":"Animal and Veterinary Research Centre (CECAV), Associate Laboratory for Animal and Veterinary Science\u2013AL4AnimalS, University of Tr\u00e1s-os-Montes and Alto Douro (UTAD), 5000-801 Vila Real, Portugal"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-6769-1844","authenticated-orcid":false,"given":"Alexandra","family":"Esteves","sequence":"additional","affiliation":[{"name":"Animal and Veterinary Research Centre (CECAV), Associate Laboratory for Animal and Veterinary Science\u2013AL4AnimalS, University of Tr\u00e1s-os-Montes and Alto Douro (UTAD), 5000-801 Vila Real, Portugal"}]},{"given":"Anabela","family":"Alves","sequence":"additional","affiliation":[{"name":"Animal and Veterinary Research Centre (CECAV), Associate Laboratory for Animal and Veterinary Science\u2013AL4AnimalS, University of Tr\u00e1s-os-Montes and Alto Douro (UTAD), 5000-801 Vila Real, Portugal"}]},{"given":"Ana Cristina","family":"Matos","sequence":"additional","affiliation":[{"name":"Research Center for Natural Resources, Environment and Society (CERNAS), Polytechnic Institute of Castelo Branco (IPCB), Av. Pedro \u00c1lvares Cabral, 12, 6000-084 Castelo Branco, Portugal"}]},{"given":"Fernanda","family":"Seixas","sequence":"additional","affiliation":[{"name":"Animal and Veterinary Research Centre (CECAV), Associate Laboratory for Animal and Veterinary Science\u2013AL4AnimalS, University of Tr\u00e1s-os-Montes and Alto Douro (UTAD), 5000-801 Vila Real, Portugal"}]},{"given":"Filipe","family":"Silva","sequence":"additional","affiliation":[{"name":"Animal and Veterinary Research Centre (CECAV), Associate Laboratory for Animal and Veterinary Science\u2013AL4AnimalS, University of Tr\u00e1s-os-Montes and Alto Douro (UTAD), 5000-801 Vila Real, Portugal"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-6330-4560","authenticated-orcid":false,"given":"Isabel","family":"Pires","sequence":"additional","affiliation":[{"name":"Animal and Veterinary Research Centre (CECAV), Associate Laboratory for Animal and Veterinary Science\u2013AL4AnimalS, University of Tr\u00e1s-os-Montes and Alto Douro (UTAD), 5000-801 Vila Real, Portugal"}]},{"given":"Luis","family":"Figueira","sequence":"additional","affiliation":[{"name":"Quality of Life in the Rural World (Q-Rural), Polytechnic Institute of Castelo Branco (IPCB), Av. 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