Neuropathology of Animal Prion Diseases

• Published in: Biomolecules (Basel, Switzerland) Vol. 11; no. 3; p. 466
• Main Authors: 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
• Format: Journal Article
• Language: English
• Published: Switzerland MDPI 21.03.2021; MDPI AG
• Subjects: animal TSE; gene PRNP; neuroinflammation; neuropathology; prion; Review; spongiform degeneration; gene PRNP; prion; animal TSE; spongiform degeneration; neuroinflammation; neuropathology
• ISSN: 2218-273X; 2218-273X
• DOI: 10.3390/biom11030466

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 ) into a misfolded pathological isoform (PrP or prion- proteinaceous infectious particle) that self-propagates by conformational conversion of PrP . Yet by an unknown mechanism, PrP can fold into different PrP 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 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).