Human consumption of beef products infected with the agent of mad-cow disease gave rise to a fatal, human neurodegenerative disease called variant Creutzfeldt-Jakob disease (vCJD) in 1996. Both mad cow and vCJD are forms of prion diseases, named after the protein thought to cause them. There is no cure, prophylaxis, or fail-safe antemortem diagnostic test for these poorly understood diseases, and a decade's worth of attempts to develop effective prion-detection tests have largely failed. Consequently, the U.S. Department of Defense launched the National Prion Research Program in 2002 with $42.5 million and asked the Institute of Medicine to provide a research agenda for the first round of grants. Advancing Prion Science is that agenda.
The recent outbreak in the Midwest of chronic wasting disease, a prion disease of deer and elk, gives the program and the IOM report added urgency. The main obstacle to developing accurate, rapid, and highly sensitive antemortem tests for prions is the lack of knowledge about the dangerous proteins and their normal cellular counterpart, concluded the IOM Committee on Transmissible Spongiform Encephalopathies: Assessment of Relevant Science.
The committee recommends that the DoD program fund basic research into the structural features of prions, the molecular mechanisms of prion replication, the mechanisms of pathogenesis, the epidemiology and natural history of prion diseases, and the physiologic function of normal prion protein. The committee also recommends several applied-research strategies: aim to detect surrogate markers of prion infection rather than prions themselves; use emerging techniques to multiply the amount of prion proteins available to test in a tissue sample; and develop emerging techniques for brain-imaging to detect the presence of prions.