title: The Priority position paper: Protecting Europe's food chain from prions.
abstract: Bovine spongiform encephalopathy (BSE) created a global European crisis in the 1980s and 90s, with very serious health and economic implications. Classical BSE now appears to be under control, to a great extent as a result of a global research effort that identified the sources of prions in meat and bone meal (MBM) and developed new animal-testing tools that guided policy. Priority ( www.prionpriority.eu ) was a European Union (EU) Framework Program 7 (FP7)-funded project through which 21 European research institutions and small and medium enterprises (SMEs) joined efforts between 2009 and 2014, to conduct coordinated basic and applied research on prions and prion diseases. At the end of the project, the Priority consortium drafted a position paper ( www.prionpriority.eu/Priority position paper) with its main conclusions. In the present opinion paper, we summarize these conclusions. With respect to the issue of re-introducing ruminant protein into the feed-chain, our opinion is that sustaining an absolute ban on feeding ruminant protein to ruminants is essential. In particular, the spread and impact of non-classical forms of scrapie and BSE in ruminants is not fully understood and the risks cannot be estimated. Atypical prion agents will probably continue to represent the dominant form of prion diseases in the near future in Europe. Atypical L-type BSE has clear zoonotic potential, as demonstrated in experimental models. Similarly, there are now data indicating that the atypical scrapie agent can cross various species barriers. More epidemiological data from large cohorts are necessary to reach any conclusion on the impact of its transmissibility on public health. Re-evaluations of safety precautions may become necessary depending on the outcome of these studies. Intensified searching for molecular determinants of the species barrier is recommended, since this barrier is key for important policy areas and risk assessment. Understanding the structural basis for strains and the basis for adaptation of a strain to a new host will require continued fundamental research, also needed to understand mechanisms of prion transmission, replication and how they cause nervous system dysfunction and death. Early detection of prion infection, ideally at a preclinical stage, also remains crucial for development of effective treatment strategies.
authors.affiliation: a CIMUS Biomedical Research Institute, University of Santiago de Compostela , Santiago de Compostela , Spain ;
authors.forename: Jesús R
authors.initials: JR
authors.lastname: Requena
authors.affiliation: b Karolinska Institute , Stockholm , Sweden ;
authors.forename: Krister
authors.initials: K
authors.lastname: Kristensson
authors.affiliation: c University of Düsseldorf , Düsseldorf , Germany ;
authors.forename: Carsten
authors.initials: C
authors.lastname: Korth
authors.affiliation: d Institut Pasteur , Paris , France ;
authors.forename: Chiara
authors.initials: C
authors.lastname: Zurzolo
authors.affiliation: e APHA , Weybridge , UK ;
authors.forename: Marion
authors.initials: M
authors.lastname: Simmons
authors.affiliation: f CISA, INIA , Madrid , Spain ;
authors.forename: Patricia
authors.initials: P
authors.lastname: Aguilar-Calvo
authors.affiliation: g University of Zürich , Zürich , Switzerland ;
authors.forename: Adriano
authors.initials: A
authors.lastname: Aguzzi
authors.affiliation: h ENVT-INRA , Toulouse , France ;
authors.forename: Olivier
authors.initials: O
authors.lastname: Andreoletti
authors.affiliation: i Norwegian Veterinary Institute , Oslo , Norway ;
authors.forename: Sylvie L
authors.initials: SL
authors.lastname: Benestad
authors.affiliation: j University of Hohenheim , Stuttgart , Germany ;
authors.forename: Reinhard
authors.initials: R
authors.lastname: Böhm
authors.affiliation: k University of Edinburgh , Edinburgh , UK ;
authors.forename: Karen
authors.initials: K
authors.lastname: Brown
authors.affiliation: l University of Barcelona , Barcelona , Spain ;
authors.forename: Byron
authors.initials: B
authors.lastname: Calgua
authors.affiliation: m IBEC , Barcelona , Spain ;
authors.forename: José Antonio
authors.initials: JA
authors.lastname: Del Río
authors.affiliation: f CISA, INIA , Madrid , Spain ;
authors.forename: Juan Carlos
authors.initials: JC
authors.lastname: Espinosa
authors.affiliation: l University of Barcelona , Barcelona , Spain ;
authors.forename: Rosina
authors.initials: R
authors.lastname: Girones
authors.affiliation: n Netherlands Cancer Institute , Amsterdam , The Netherlands ;
authors.forename: Sue
authors.initials: S
authors.lastname: Godsave
authors.affiliation: j University of Hohenheim , Stuttgart , Germany ;
authors.forename: Ludwig E
authors.initials: LE
authors.lastname: Hoelzle
authors.affiliation: o Friedrich Löffler Institut , Insel Reims , Germany ;
authors.forename: Michael R
authors.initials: MR
authors.lastname: Knittler
authors.affiliation: p Prionics , Zürich , Switzerland ;
authors.forename: Franziska
authors.initials: F
authors.lastname: Kuhn
authors.affiliation: q SISSA , Trieste , Italy ;
authors.forename: Giuseppe
authors.initials: G
authors.lastname: Legname
authors.affiliation: r University of Maastricht , Maastricht , The Netherlands ;
authors.forename: Paul
authors.initials: P
authors.lastname: Laeven
authors.affiliation: k University of Edinburgh , Edinburgh , UK ;
authors.forename: Neil
authors.initials: N
authors.lastname: Mabbott
authors.affiliation: s Medical University of Slovakia , Bratislava , Slovakia ;
authors.forename: Eva
authors.initials: E
authors.lastname: Mitrova
authors.affiliation: c University of Düsseldorf , Düsseldorf , Germany ;
authors.forename: Andreas
authors.initials: A
authors.lastname: Müller-Schiffmann
authors.affiliation: g University of Zürich , Zürich , Switzerland ;
authors.forename: Mario
authors.initials: M
authors.lastname: Nuvolone
authors.affiliation: t The Maastricht Multimodal Molecular Imaging Institute, University of Maastricht , Maastricht , The Netherlands ;
authors.forename: Peter J
authors.initials: PJ
authors.lastname: Peters
authors.affiliation: p Prionics , Zürich , Switzerland ;
authors.forename: Alex
authors.initials: A
authors.lastname: Raeber
authors.affiliation: u SMP GmbH , Tübingen , Germany ;
authors.forename: Klaus
authors.initials: K
authors.lastname: Roth
authors.affiliation: v Universitätmedizin Göttingen , Göttingen , Germany ;
authors.forename: Matthias
authors.initials: M
authors.lastname: Schmitz
authors.affiliation: p Prionics , Zürich , Switzerland ;
authors.forename: Björn
authors.initials: B
authors.lastname: Schroeder
authors.affiliation: g University of Zürich , Zürich , Switzerland ;
authors.forename: Tiziana
authors.initials: T
authors.lastname: Sonati
authors.affiliation: o Friedrich Löffler Institut , Insel Reims , Germany ;
authors.forename: Lothar
authors.initials: L
authors.lastname: Stitz
authors.affiliation: w The Hebrew University , Jerusalem ;
authors.forename: Albert
authors.initials: A
authors.lastname: Taraboulos
authors.affiliation: f CISA, INIA , Madrid , Spain ;
authors.forename: Juan María
authors.initials: JM
authors.lastname: Torres
authors.affiliation: u SMP GmbH , Tübingen , Germany ;
authors.forename: Zheng-Xin
authors.initials: ZX
authors.lastname: Yan
authors.affiliation: v Universitätmedizin Göttingen , Göttingen , Germany ;|x German Center for Neurodegenerative Diseases (DZNE) , Göttingen , Germany.
authors.forename: Inga
authors.initials: I
authors.lastname: Zerr
mesh_terms: D000821:Animal Feed / Q000009:adverse effects; D000818:Animals; D002417:Cattle; D042241:Early Diagnosis; D016643:Encephalopathy, Bovine Spongiform / Q000175:diagnosis / Q000453:epidemiology / Q000517:prevention & control / Q000635:transmission; D005060:Europe / Q000453:epidemiology; D020387:Food Chain; D006801:Humans; D017096:Prion Diseases / Q000175:diagnosis / Q000453:epidemiology* / Q000517:prevention & control* / Q000635:transmission; D011328:Prions / Q000032:analysis* / Q000302:isolation & purification / Q000378:metabolism / Q000472:pathogenicity; D012608:Scrapie / Q000175:diagnosis / Q000453:epidemiology / Q000517:prevention & control / Q000635:transmission
chemical_list: D011328:Prions
keywords: BSE; CJD; atypical BSE; atypical scrapie; prion; scrapie