Chronic wasting disease in Europe: new strains on the horizon

Abstract Prion diseases are fatal neurodegenerative disorders with known natural occurrence in humans and a few other mammalian species. The diseases are experimentally transmissible, and the agent is derived from the host-encoded cellular prion protein (PrPC), which is misfolded into a pathogenic c...

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Autores principales:	Michael Andreas Tranulis, Dolores Gavier-Widén, Jørn Våge, Maria Nöremark, Sirkka-Liisa Korpenfelt, Maria Hautaniemi, Laura Pirisinu, Romolo Nonno, Sylvie Lafond Benestad
Formato:	article
Lenguaje:	EN
Publicado:	BMC 2021
Materias:	CWD Deer Fennoscandia Moose Nordic countries Prion disease Veterinary medicine SF600-1100
Acceso en línea:	https://doaj.org/article/6e3f41bff80148549f3a1e5a6cc3e46e
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spelling	oai:doaj.org-article:6e3f41bff80148549f3a1e5a6cc3e46e2021-11-28T12:29:19ZChronic wasting disease in Europe: new strains on the horizon10.1186/s13028-021-00606-x1751-0147https://doaj.org/article/6e3f41bff80148549f3a1e5a6cc3e46e2021-11-01T00:00:00Zhttps://doi.org/10.1186/s13028-021-00606-xhttps://doaj.org/toc/1751-0147Abstract Prion diseases are fatal neurodegenerative disorders with known natural occurrence in humans and a few other mammalian species. The diseases are experimentally transmissible, and the agent is derived from the host-encoded cellular prion protein (PrPC), which is misfolded into a pathogenic conformer, designated PrPSc (scrapie). Aggregates of PrPSc molecules, constitute proteinaceous infectious particles, known as prions. Classical scrapie in sheep and goats and chronic wasting disease (CWD) in cervids are known to be infectious under natural conditions. In CWD, infected animals can shed prions via bodily excretions, allowing direct host-to-host transmission or indirectly via prion-contaminated environments. The robustness of prions means that transmission via the latter route can be highly successful and has meant that limiting the spread of CWD has proven difficult. In 2016, CWD was diagnosed for the first time in Europe, in reindeer (Rangifer tarandus) and European moose (Alces alces). Both were diagnosed in Norway, and, subsequently, more cases were detected in a semi-isolated wild reindeer population in the Nordfjella area, in which the first case was identified. This population was culled, and all reindeer (approximately 2400) were tested for CWD; 18 positive animals, in addition to the first diagnosed case, were found. After two years and around 25,900 negative tests from reindeer (about 6500 from wild and 19,400 from semi-domesticated) in Norway, a new case was diagnosed in a wild reindeer buck on Hardangervidda, south of the Nordfjella area, in 2020. Further cases of CWD were also identified in moose, with a total of eight in Norway, four in Sweden, and two cases in Finland. The mean age of these cases is 14.7 years, and the pathological features are different from North American CWD and from the Norwegian reindeer cases, resembling atypical prion diseases such as Nor98/atypical scrapie and H- and L-forms of BSE. In this review, these moose cases are referred to as atypical CWD. In addition, two cases were diagnosed in red deer (Cervus elaphus) in Norway. The emergence of CWD in Europe is a threat to European cervid populations, and, potentially, a food-safety challenge, calling for a swift, evidence-based response. Here, we review data on surveillance, epidemiology, and disease characteristics, including prion strain features of the newly identified European CWD agents.Michael Andreas TranulisDolores Gavier-WidénJørn VågeMaria NöremarkSirkka-Liisa KorpenfeltMaria HautaniemiLaura PirisinuRomolo NonnoSylvie Lafond BenestadBMCarticleCWDDeerFennoscandiaMooseNordic countriesPrion diseaseVeterinary medicineSF600-1100ENActa Veterinaria Scandinavica, Vol 63, Iss 1, Pp 1-15 (2021)
institution	DOAJ
collection	DOAJ
language	EN
topic	CWD Deer Fennoscandia Moose Nordic countries Prion disease Veterinary medicine SF600-1100
spellingShingle	CWD Deer Fennoscandia Moose Nordic countries Prion disease Veterinary medicine SF600-1100 Michael Andreas Tranulis Dolores Gavier-Widén Jørn Våge Maria Nöremark Sirkka-Liisa Korpenfelt Maria Hautaniemi Laura Pirisinu Romolo Nonno Sylvie Lafond Benestad Chronic wasting disease in Europe: new strains on the horizon
description	Abstract Prion diseases are fatal neurodegenerative disorders with known natural occurrence in humans and a few other mammalian species. The diseases are experimentally transmissible, and the agent is derived from the host-encoded cellular prion protein (PrPC), which is misfolded into a pathogenic conformer, designated PrPSc (scrapie). Aggregates of PrPSc molecules, constitute proteinaceous infectious particles, known as prions. Classical scrapie in sheep and goats and chronic wasting disease (CWD) in cervids are known to be infectious under natural conditions. In CWD, infected animals can shed prions via bodily excretions, allowing direct host-to-host transmission or indirectly via prion-contaminated environments. The robustness of prions means that transmission via the latter route can be highly successful and has meant that limiting the spread of CWD has proven difficult. In 2016, CWD was diagnosed for the first time in Europe, in reindeer (Rangifer tarandus) and European moose (Alces alces). Both were diagnosed in Norway, and, subsequently, more cases were detected in a semi-isolated wild reindeer population in the Nordfjella area, in which the first case was identified. This population was culled, and all reindeer (approximately 2400) were tested for CWD; 18 positive animals, in addition to the first diagnosed case, were found. After two years and around 25,900 negative tests from reindeer (about 6500 from wild and 19,400 from semi-domesticated) in Norway, a new case was diagnosed in a wild reindeer buck on Hardangervidda, south of the Nordfjella area, in 2020. Further cases of CWD were also identified in moose, with a total of eight in Norway, four in Sweden, and two cases in Finland. The mean age of these cases is 14.7 years, and the pathological features are different from North American CWD and from the Norwegian reindeer cases, resembling atypical prion diseases such as Nor98/atypical scrapie and H- and L-forms of BSE. In this review, these moose cases are referred to as atypical CWD. In addition, two cases were diagnosed in red deer (Cervus elaphus) in Norway. The emergence of CWD in Europe is a threat to European cervid populations, and, potentially, a food-safety challenge, calling for a swift, evidence-based response. Here, we review data on surveillance, epidemiology, and disease characteristics, including prion strain features of the newly identified European CWD agents.
format	article
author	Michael Andreas Tranulis Dolores Gavier-Widén Jørn Våge Maria Nöremark Sirkka-Liisa Korpenfelt Maria Hautaniemi Laura Pirisinu Romolo Nonno Sylvie Lafond Benestad
author_facet	Michael Andreas Tranulis Dolores Gavier-Widén Jørn Våge Maria Nöremark Sirkka-Liisa Korpenfelt Maria Hautaniemi Laura Pirisinu Romolo Nonno Sylvie Lafond Benestad
author_sort	Michael Andreas Tranulis
title	Chronic wasting disease in Europe: new strains on the horizon
title_short	Chronic wasting disease in Europe: new strains on the horizon
title_full	Chronic wasting disease in Europe: new strains on the horizon
title_fullStr	Chronic wasting disease in Europe: new strains on the horizon
title_full_unstemmed	Chronic wasting disease in Europe: new strains on the horizon
title_sort	chronic wasting disease in europe: new strains on the horizon
publisher	BMC
publishDate	2021
url	https://doaj.org/article/6e3f41bff80148549f3a1e5a6cc3e46e
work_keys_str_mv	AT michaelandreastranulis chronicwastingdiseaseineuropenewstrainsonthehorizon AT doloresgavierwiden chronicwastingdiseaseineuropenewstrainsonthehorizon AT jørnvage chronicwastingdiseaseineuropenewstrainsonthehorizon AT marianoremark chronicwastingdiseaseineuropenewstrainsonthehorizon AT sirkkaliisakorpenfelt chronicwastingdiseaseineuropenewstrainsonthehorizon AT mariahautaniemi chronicwastingdiseaseineuropenewstrainsonthehorizon AT laurapirisinu chronicwastingdiseaseineuropenewstrainsonthehorizon AT romolononno chronicwastingdiseaseineuropenewstrainsonthehorizon AT sylvielafondbenestad chronicwastingdiseaseineuropenewstrainsonthehorizon
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Chronic wasting disease in Europe: new strains on the horizon

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