Commission to promote prion research

The European Commission last week launched a new research network
which it hopes will become the world's leading resource on prion
diseases such as BSE - evidence that while 'mad cow' fears might
have diminished, the threat is still considered to be a major one.

The network was officially launched last Friday by European Research Commissioner Philippe Busquin, and consists of 52 laboratories in 20 countries, bringing together 90 per cent of the European research teams working on BSE (Bovine Spongiform Encephalopathy), vCJD (variant Creutzfeldt Jakob Disease, the human form of BSE), scrapie (a sheep prion disease) and other types of prion diseases.

The European Union research budget will provide €14.4 million over five years to the network, and a new prion research facility will also be launched at the CEA (Commissariat à l'Energie Atomique), a major multidisciplinary research organisation in France and the institution co-ordinating the NeuroPrion network.

"The European Commission has made a sustained effort to tackle the BSE crisis on several fronts,"​ said Busquin. "This has included a special action plan for research, launched in 1996, which rapidly mobilised €50 million worth of research involving more than 120 laboratories throughout Europe. As part of the European Research Area, the NeuroPrion network of excellence is the logical next step. The network will bring together Europe's top experts to tackle prion diseases in terms of prevention, control, treatment and risk analysis."

Transmissible Spongiform Encephalopathy (TSE) is the generic name for the spectrum of diseases which are all thought to be caused by the abnormal folding of the prion protein. TSEs include BSE, vCJD, scrapie and other forms, such as Chronic Wasting Disease in deer and elk. BSE was first identified in 1986.

Since then, 180,000 cases of BSE have been diagnosed in the UK alone. Only four out of the 25 EU Member States have not yet declared any cases. A number of countries outside Europe have also found cases, making this a global disease. The impact of BSE on European society has been far reaching with a devastating impact on vCJD patients and their families. Some 146 cases of probable and confirmed vCJD have been reported.

BSE has affected the entire beef food chain, from producer to consumer. Farmers have watched their animals slaughtered in their thousands and seen their livelihoods jeopardised. A European Association of Animal Production report has estimated the cost of BSE to the states of the 15 member EU at more than €90 billion. In addition, the BSE crisis has had a significant impact on public trust in government and governmental scientific advice.

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 an infected animal.

The European NeuroPrion network has been established to help scientists better understand how prion modifications occur, how they develop in animals, how they are transferred to humans and how they affect human health and in particular the brain and nerve cells. It aims to create a research infrastructure that will attract private investment, during and beyond the five-year funding allocated by the EU. The group has identified areas where novel, applied research is needed, and areas where greater co-ordination of research would be beneficial.

The most urgent research needs concern diagnosing prion diseases well in advance. Detection methods, involving the analysis of easily accessible body fluids early on in the incubation period, will be developed by the NeuroPrion network. 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 and in animals will enhance food safety even further.

But surveillance and the analysis of risk from prion diseases both require international co-ordination. NeuroPrion encompasses all national surveillance centres for vCJD and will also link animal surveillance. Institutes will share training, exchange staff and have access to a specially designed website, while 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, since they can be engineered to carry prion genes from other species and can be used to model the disease over a much shorter timeframe.

Related topics R&D