Prion Disease: Overview
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.
Identified Prion Diseases
Listed below are the prion diseases identified to date. CDC
does not currently offer information on every prion disease listed below.
Human Prion Diseases
- Creutzfeldt-Jakob Disease (CJD)
- Variant Creutzfeldt-Jakob Disease (vCJD)
- Gerstmann-Straussler-Scheinker Syndrome
- Fatal Familial Insomnia
- Kuru
Animal Prion Diseases
- Bovine Spongiform Encephalopathy (BSE)
- Chronic Wasting Disease (CWD)
- Scrapie
- Transmissible mink encephalopathy
- Feline spongiform encephalopathy
- Ungulate spongiform encephalopathy
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