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Mad cow disease, or bovine spongiform encephalopathy (BSE), cost Europe dearly – €2.7 billion were spent buying cattle from farmers for destruction in 2000-2001 alone. The risk from the human form of BSE, known as variant Creutzfeldt- Jakob disease (vCJD), is still uncertain. BSE has a fiveyear ‘silent’ incubation period in cattle; in humans, the infection can remain silent for up to 40 years. Sheep were also exposed to the infectious agent via contaminated feed. Although no naturally occurring case of sheep BSE has been found, questions remain. The discovery of cases outside Europe also raises concerns for the rest of the world. Thus, whilst European cases of cattle BSE are on the decrease, we cannot be complacent.

These and similar diseases, such as scrapie in sheep, are thought to be caused by self-replicating malformed variants of proteins, the normal forms of which are present in many animals. This theory, known as the ‘prion hypothesis’, was very controversial when it was first proposed, since all other infectious diseases are caused by organisms which contain genetic material (DNA or RNA). Whilst many scientists now support the prion hypothesis, there remains significant debate on the exact nature of these diseases.

What is certain is that the diseases are difficult to diagnose in their early stages and are, as yet, invariably fatal. The NeuroPrion Network of Excellence incorporates most European research groups studying prions and aims to become the leading international task force on prion biology.


The exact function of normal prion protein is not known. In disease, the malformed protein accumulates in the brain causing dementia. A number of animal species may carry different genes encoding subtly different forms of prion protein, some of which make them more susceptible to developing disease. This can occur spontaneously, or, as in the case of BSE, as a result of being exposed to modified prions from a sick animal.

The network involves 52 leading research groups, accounting for more than 80% of European prion research. It aims to create a research infrastructure that will attract private investment, during and beyond the five years funding allocated by the EU. The group has identified areas where novel, applied research is needed, and areas where greater coordination of research would be beneficial.


The most urgent research need concerns diagnosing prion diseases well before death. It is hoped that detection methods analysing easily accessible body fluids early on in the incubation period will be developed. Such tests could be used to screen both people and animals. Early diagnosis in humans will increase the likelihood of the success of therapeutic interventions; in animals, it will enhance food safety even further.


Surveillance and the analysis of risk from prion diseases both require international coordination. NeuroPrion encompasses all national surveillance centres for vCJD and will link animal surveillance also. Institutes will share training, exchange staff and have access to a specially designed website. Tissue and fluid banks will also be shared and standard methods agreed. Genetically altered mice strains held by different institutions will be made available to partners. These are a vital tool as they can be engineered to carry prion genes from other species, and can be used to model the disease over a much shorter timeframe.

The network will interact with the greater scientific community and the public at an annual world congress on prions.

List of Partners

  • Commissariat à l’Energie Atomique (France)
  • Institut National de le Recherche Agronomique (France)
  • Agence Française de Sécurité Sanitaire des Aliments (France)
  • Centre National de la Recherche Scientifique (France)
  • Institut National de la Santé et de la Recherche Médicale (France)
  • Association pour la Recherche et la Développement des Méthodes et Processus Industriels (France)
  • University of Edinburgh (UK)
  • Department for Environment, Food and Rural Affairs (UK)
  • Institute for Animal Health (UK)
  • Health Protection Agency (UK)
  • Instituto Nacional de Investigación y Tecnologiá Agraria y Alimentaria (Spain)
  • Institut Neuropatologica, Hospital Universitari Bellvitge (Spain)
  • Universidad de Zaragoza (Spain)
  • Centre for Research and Technology – Hellas/Institute for Agrobiotechnology (Greece)
  • Medical Research Council (UK)
  • Ecole Nationale Vétérinaire d’Alfort (France)
  • Faculty of Mathematics, Physics and Computer Sciences, Comenius University (Slovakia)
  • Royal Veterinary College (UK)
  • Moredun Research Institute, Edinburgh (UK)
  • National University of Ireland, Dublin (Ireland)
  • Heinrich-Heine-Universität Düsseldorf (Germany)
  • Ludwig-Maximilians-Universität (Germany)
  • Federal Research Centre for Virus Diseases of Animals (Germany)
  • Robert Koch-Institut (Germany)
  • Centraal Institut voor DierziekteControle-Lelystad, part of DLO foundation (The Netherlands)
  • Istituto Nazionale Neurologico Carlo Besta (Italy)
  • Istituto Supreriore di Sanità (Italy)
  • Istituto di Richerche Farmacologiche “Mario Negri” (Italy)
  • Cea-Centro Encefalopatie Animali Istituto Zooprofilattico Sperimentale del Piemonte, Liguria e Vallee d’Aosta (Italy)
  • Università di Bologna (Italy)
  • Institut für Neurologie, Universität Wien (Austria)
  • University of Zurich (Switzerland)
  • National Veterinary Institute (Norway)
  • European Commission Joint Research Centre (Belgium)
  • Hadassah Medical Organisation (Israel)
  • University of Southampton (UK)
  • Roslin Institute (UK)
  • ID-Lelystad, Institut voor Dierhouderij en Diergezondheid (The Netherlands)
  • Technische Universität München (Germany)
  • Institute of Zoo and Wildlife Research, Berlin (Germany)
  • Norwegian School of Veterinary Science (Norway)
  • National Veterinary Institute (Sweden)
  • Karolinska Institute (Sweden)
  • Danish Veterinary Institute (Denmark)
  • National Veterinary and Food Research Institute (Finland)
  • Institute for Experimental Pathology (Iceland)
  • Consejo Superior de Investigaciones Cientificas (Spain)
  • Medical University of Lodz (Poland)
  • Université René Descartes (Paris 5) (France)
  • Westfälische Wilhelms- University, University Hospital Münster (Germany)
  • Laboratorio Nacional de Investigacao Veterinaria (Portugal)
  • University of Verona (Italy)
Full title:
Prevention, control and management of prion diseases
Contract n°:
Project co-ordinator:
Jean-Philippe DESLYS, Commissariat à l’Energie Atomique,
EC Scientific Officer:
Laurence Moreau,
EU contribution:
€ 14.4M
Network of Excellence

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Last update: 06 December 2007 | Top