About Prion Diseases
||Cattle at a trough, and a buck deer in the wild. (Courtesy Ermias Belay)
Prion diseases or transmissible spongiform encephalopathies (TSEs) are a family of rare progressive neurodegenerative disorders that affect both humans and animals. They are distinguished by long incubation periods, characteristic spongiform changes associated with neuronal loss, and a failure to induce inflammatory response.
The causative agents of TSEs are believed to be prions. The term "prions" refers to abnormal, pathogenic agents that are transmissible and are able to induce abnormal folding of specific normal cellular proteins called prion proteins that are found most abundantly in the brain. The functions of these normal prion proteins are still not completely understood. The abnormal folding of the prion proteins leads to brain damage and the characteristic signs and symptoms of the disease. Prion diseases are usually rapidly progressive and always fatal.
A List of Prion Diseases
Listed below are the prion diseases identified to date. Click the linked diseases to go to their respective topic sites. CDC does not currently offer information here on every prion disease listed.
Human Prion Diseases
Animal Prion Diseases
Links to Organizations Outside CDC
National Prion Disease Pathology Surveillance Center
(From the Division of Neuropathology, Case Western Reserve University. National CJD surveillance system established in collaboration with CDC.)
BSE/TSE Action Plan of the Department of Health and Human Services (DHHS)
The BSE/TSE Action Plan of DHHS has four major components:
- Surveillance for human disease is primarily the responsibility of CDC.
- Protection is primarily the responsibility of the Food and Drug Administration (FDA).
- Research is primarily the responsibility of the National Institutes of Health (NIH).
- Oversight is primarily the responsibility of the Office of the Secretary of DHHS.
Press Release: HHS Launches Expanded Plan to Combat "Mad Cow Disease"
On DHHS site
The Public Health Impact of Prion Diseases
Belay E., Schonberger L. Annu. Rev. Public Health 2005;26:191-212
PDF format (198 KB/25 pages)
Transmissible Spongiform Encephalopathies in Humans
Belay E. Annu. Rev. Microbiol. 1999;53:283-314
PDF format (208 KB/32 pages)
WHO Infection Control Guidelines for Transmissible Spongiform Encephalopathies: Report of a WHO Consultation, Geneva, Switzerland, 23-26 March 1999
(From the World Health Organization)
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