Advancing Prion Science: Guidance for the National Prion Research Program

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National Academies Press, Apr 4, 2004 - Medical - 288 pages
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In Advancing Prion Science, the Institute of Medicineā€™s Committee on Transmissible Spongiform Encephalopathies Assessment of Relevant Science recommends priorities for research and investment to the Department of Defenseā€™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 prionsā€™ normal cellular isoform. Advancing Prion Science provides the first comprehensive reference on present knowledge about all aspects of TSEsā€"from basic science to the U.S. research infrastructure, from diagnostics to surveillance, and from prevention to treatment.

 

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Contents

EXECUTIVE SUMMARY
SUMMARY
Origins of This Study
Prions and PrPˢc
Basic Biomedical Research
TSE Diagnostics
Testing Blood for Evidence of TSEs
Surveillance for TSEs in the United States
Research Recommendations for TSE Diagnostics
References
TESTING BLOOD FOR EVIDENCE OF THE AGENTS OF TRANSMISSIBLE SPONGIFORM ENCEPHALOPATHIES
Animal Studies to Assess TSE Infectivity of Blood
Risk of HumantoHuman Transmission of TSE Agents by Transfusion and Transplant
Blood Tests for TSE Agents
References
SURVEILLANCE FOR TRANSMISSIBLE SPONGIFORM ENCEPHALOPATHIES IN THE UNITED STATES

Assessment of Strategies to Prevent and Treat TSEs
Research Infrastructure
The Risk of TSEs to the US Military
Conclusion
References
INTRODUCTION
Charge to the Committee
References
PRION DISEASES AN OVERVIEW
Origins and Development of Prion Science
The Nature of Prions and Prion Protein
The Epidemic of BSE and the Emergence of vCJD
Global Impact of BSE and vCJD
The Spread of Chronic Wasting Disease in the United States
Unique Challenges in Conducting TSE Research
References
BASIC BIOMEDICAL RESEARCH ON TRANSMISSIBLE SPONGIFORM ENCEPHALOPATHIES
Structural features of Prions
Molecular Mechanisms of Prion Replication
Mechanisms of TSE Pathogenesis
Physiological Function of PrPc
References
DIAGNOSTICS FOR TRANSMISSIBLE SPONGIFORM
Clinical Diagnostics
Current Laboratory Diagnostics
Newer Experimental Diagnostics for Laboratory Use
US Surveillance for Human TSEs
US Surveillance for TSEs in Animals
Essential Research to Improve US Capabilities to Conduct Surveillance for TSEs
References
ASSESSMENT OF STRATEGIES TO PREVENT AND TREAT TRANSMISSIBLE SPONGIFORM ENCEPHALOPATHIES
Measures to Prevent the CWD Agent from Entering the US Food Chain
Preventing TSE Transmission Through Blood Blood Derivatives and Transplanted Tissues
Inactivation of Prions on Surfaces and in the Environment
Vaccination as a Preventive Strategy
Progress in Therapy
References
INFRASTRUCTURE FOR RESEARCH ON TRANSMISSIBLE SPONGIFORM ENCEPHALOPATHIES
Need for Consistent ScienceBased Standards for Biologic Safety Levels in TSE Laboratories
Need for Standardized Reagents and Materials
Opportunities for International Collaboration
References
RISK OF TRANSMISSIBLE SPONGIFORM ENCEPHALOPATHIES TO THE US MILITARY
Risk of Exposure to Beef Products Containing BSE Infectivity
Risk of TSE Infection from Blood Products
Summary of Overall Risk
References
APPENDIXES
B BIOGRAPHICAL SKETCHES
GLOSSARY
Copyright

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