The MRC will spend £1.5 million ($2.5 million) a year for the next five years on a new London research center to study Creutzfeldt Jakob Disease (CJD) and other prion diseases. Although it will carry out basic research, such as the study of prion protein structure and disease pathogenesis, a top priority for the new center will be to develop tests for CJD and 'new variant' CJD (nvCJD) in human tissue samples. Such tests, it is hoped, will provide an early warning of any imminent large-scale health problem that some fear could still result from the BSE epidemic among cattle in the mid-1980s.

The UK government has spent £2.5 billion to slaughter about 30 million cattle and estimates that an additional £1 billion will be needed to dispose of more animals by the year 2000.

Thirty people have died so far from nvCJD and until recently, its diagnosis could only be confirmed postmortem from a brain biopsy. But researchers have discovered that the abnormal prion precursor can be detected in appendix and tonsil tissue removed during surgery from patients later found to suffer from CJD. Consequently, the new unit plans to carry out anonymous screening of tonsils stored at hospitals throughout the UK to estimate the extent of the disease's spread. If testing reveals cases of nvCJD, Britain's Chief Medical Office, Sir Kenneth Calman, says it may be necessary to notify individuals.

The long incubation period of nvCJD makes it difficult to determine the potential size of the health problem. "If you had a test that you felt was sensitive all the way through the incubation period, then an obvious step is to undertake some sort of anonymous screening," says Azra Ghani, a statistician at the University of Oxford, but she warns that even large-scale screening will carry a high level of uncertainty if the test is unable to detect the early stages of an infection (The Lancet 352, 1353; 1998).

John Collinge, head of the neurogenetics department at the Imperial College School of Medicine at St. Mary's Hospital and a prion expert, has been appointed director of the new unit. Charles Weissmann, a molecular biologist at the University of Zurich, is scheduled to join the project in March of 1999, and the unit, which is intended to comprise 60 staff, may eventually move into its own facility separate from its initial base at St Mary's Hospital. Collinge believes that "inevitably this will be a unit which the government can turn to for answers to particular questions."

John Collinge

Whether the government will listen is another matter. Evidence that has emerged from the ongoing, and increasingly acrimonious, BSE inquiry suggests that the Ministry of Agriculture, Food and Fisheries ignored the advice of scientists in the early 1990s (Nature Med. 4, 135; 1998) and neglected to inform Calman of the potential danger. Specific accusations are likely to be formulated in the second phase of the inquiry, scheduled to begin in February of 1999.