Prion Diseases

Prion diseases or transmissible spongiform encephalopathies (TSEs) are a family of progressive neurodegenerative disorders in humans and animals. The causative agents of TSEs are believed to be prions. Prion proteins are normal cellular proteins that are found mostly in the brain. The function of these proteins are still not completely understood. The term “prions” refers to abnormal, pathogenic prion proteins that are transmissible. They are able to induce abnormal folding of normal cellular prion proteins. As the amount of abnormal prion protein grows, it becomes hard to break down, causing brain degeneration and neurologic disease. Prion diseases are always fatal and have long incubation periods that are often measured in years.

For additional information about prion diseases, including Creutzfeldt-Jakob disease (CJD), please see below.

• Creutzfeldt-Jakob Disease (CJD)