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Prion Diseases

About Prion Diseases

Collage: Cattle at a trough and a buck deer in the wild.
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.)

Resources

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 icon
PDF format (198 KB/25 pages)

Transmissible Spongiform Encephalopathies in Humans
Belay E. Annu. Rev. Microbiol. 1999;53:283-314
PDF format icon
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)

Reference in this website to any specific commercial products, process, service, manufacturer, or company does not constitute its endorsement or recommendation by the U.S. Government or CDC. CDC is not responsible for the contents of any "off-site" web page referenced from this server.

Date: December 10, 2012
Content source: Centers for Disease Control and Prevention
National Center for Emerging and Zoonotic Infectious Diseases (NCEZID)
Division of High-Consequence Pathogens and Pathology (DHCPP)
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Bovine Spongiform Encephalopathy (BSE)

Chronic Wasting Disease (CWD)

Creutzfeldt-Jakob Disease, Classic (CJD)

Variant Creutzfeldt-Jakob Disease (vCJD)

Contact CDC

  • Centers for Disease Control and Prevention
    National Center for Emerging and Zoonotic Infectious Diseases (NCEZID)
    Division of High-Consequence Pathogens and Pathology (DHCPP)


    1 (800) CDC-INFO (232-4636)
    TTY: 1 (888) 232-6348

    E-mail:
    prion@cdc.gov

 
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