In Advancing Prion Science, the IOM Committee on Transmissible Spongiform Encephalopathies: Assessment of Relevant Science recommends priorities for research and investment to the Department of Defense's National Prion Research Program (NPRP). Transmissible spongiform encephalopathies (TSEs), also called prion diseases, are invariably fatal neurodegenerative infectious diseases that include bovine spongiform encephalopathy (commonly called mad cow disease), chronic wasting disease, Creutzfeldt-Jakob disease, and scrapie. Unlike all other known infectious diseases, TSE infectivity appears to be associated with an abnormally folded protein called a prion.
To develop antemortem diagnostics or therapies for TSEs, the committee concludes, NPRP should invest in basic research specifically to elucidate the structural features of prions, the molecular mechanisms of prion replication, the mechanisms of TSE pathogenesis, and the physiological function of prions' normal cellular isoform. The committee makes a number of other research recommendations; these include the expansion of research into the natural history, prevalence, distribution, host susceptibility, host range, and exposure and transmission characteristics of TSEs--especially chronic wasting disease, a growing epidemic among U.S. deer and elk.
Advancing Prion Science provides the first comprehensive reference on present knowledge about all aspects of TSEs--from basic science to the U.S. research infrastructure; from diagnostics to surveillance; and from prevention to treatment. The report also explains the controversy in scientific circles over the very meaning of the word prion, reflecting of the newness of this area in the field of infectious diseases.