Prion Protein
About Prion Diseases
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.
Prion
Research suggests that TSEs are caused by an abnormal version of a protein called a prion (prion is short for proteinaceous infectious particle). Prion proteins occur in both a normal form, which is a harmless protein found in the body's cells, and in an infectious form, which causes disease.
The harmless and infectious forms of the prion protein are nearly identical, but the infectious form takes on a different folded shape from the normal protein.
In the infectious form, a prion is an abnormal, transmissible agent that is able to induce abnormal folding of normal cellular prion proteins in the brain, leading to brain damage and the characteristics signs and symptoms of the disease. Prion diseases are usually rapidly progressive and always fatal.
A List of Prion Diseases Identified to Date
Human Prion Diseases
1) Creutzfeldt-Jakob Disease (CJD)
2) variant Creutzfeldt-Jakob Disease (vCJD)
3) Gerstmann-Straussler-Scheinker Syndrome (GSS)
4) Fatal Familial Insomnia (FFI)
5) Kuru
Animal Prion Diseases
1) Bovine Spongiform Encephalopathy (BSE) in cattle, known as mad cow disease
2) Chronic Wasting Disease (CWD) affecting mule deer, elk, white tailed deer
3) Scrapie affecting sheep, goats, mouflon
4) Transmissible Mink Encephalopathy afftecting mink
5) Feline Spongiform Encephalopathy afftecting domestic cats and captive exotic felines
6) Exotic Ungulate spongiform encephalopathy (EUE) afftecting nyala, greater kudu, oryx, gemsbok, cape elk
National Prion Disease Pathology Surveillance Center at www.cjdsurveillance.com/ - From the Division of Neuropathology, Case Western Reserve University. National CJD surveillance system established in collaboration with CDC (Centers for Disease Control and Prevention)
References:
January 2006
www.cdc.gov/ncidod/dvrd/prions/
National Center for Infectious Diseases
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