Advancing Prion Science: Guidance for the National Prion Research Program -- Interim ReportIn Advancing Prion Science, the Institute of Medicine's 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, scrapie, and Creutzfeldt-Jakob disease. To develop antemortem diagnostics or therapies for TSEs, the committee concludes that 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. 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. This report summarizes the progress thus far. |
Contents
EXECUTIVE SUMMARY | 1 |
INTRODUCTION | 17 |
PRION DISEASES AND THEIR CHALLENGES | 23 |
Copyright | |
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abnormal Academy of Sciences amount of PrPS animals antibody aptamers assays Assessment of Relevant associated with prion beef products bind bioassay biological bovine spongiform encephalopathy cattle chronic wasting disease clinical Committee on Transmissible Creutzfeldt-Jakob disease detect prions Determine diagnostic tests digestion Epidemiology Europe European funding Gajdusek Gambetti Health Identify incubation period investigators Journal kuru laboratory methods mice molecular molecules multiphoton microscopy National Academy National Prion Research NPRP nucleic acids Parchi pathogenesis patients percent plaques prion detection prion disease prion protein prion replication prion strains prions in human PRNP proteinase proteinase K Prpsc Prusiner SB reagents Recommendation repositories risk sCJD sCJD and vCJD scrapie scrapie agent sensitive specific spongiform encephalopathies TSES structure surveillance techniques Tests for TSES transfusion transgenic Transmissible Spongiform Encephalopathies TSE diagnostics TSE research U.S. Department U.S. forces U.S. military United Kingdom variant Creutzfeldt-Jakob disease vCJD Veterinary Virology vitro Western blotting