Prions are abnormal, pathogenic agents that are transmissible and can induce abnormal folding of specific normal cellular proteins called prion proteins that are found most abundantly in the brain. Prion diseases, also known as transmissible spongiform encephalopathies (TSEs), are a family of rare progressive neurodegenerative disorders that affect both humans and animals. Prion diseases are caused by the abnormal folding of prion proteins, which leads to brain damage and the characteristic signs and symptoms of the disease. Prion diseases are usually rapidly progressive and always fatal.
Some of the identified prion diseases include Creutzfeldt-Jakob Disease (CJD), Variant Creutzfeldt-Jakob Disease (vCJD), Gerstmann-Straussler-Scheinker Syndrome, Fatal Familial Insomnia, Kuru, Bovine Spongiform Encephalopathy (BSE), Chronic Wasting Disease (CWD), Scrapie, Transmissible mink encephalopathy, Feline spongiform encephalopathy, and Ungulate spongiform encephalopathy.
Prion diseases can affect both humans and animals and are sometimes spread to humans by infected meat products. The most common form of prion disease that affects humans is Creutzfeldt-Jakob disease (CJD). Prion diseases are rare, with about 300 cases reported each year in the U.S.
Prion diseases occur when normal prion protein, found on the surface of many cells, becomes abnormal and clumps in the brain, causing brain damage. Prions form abnormal aggregates of proteins called amyloids, which accumulate in infected tissue and are associated with tissue damage and cell death. Prions are a type of intrinsically disordered protein, which change their conformation unless they are bound to a specific partner such as another protein.
Despite more than a decade’s worth of research, many aspects of prion diseases still remain a mystery. Prion diseases are not curable, though symptoms can be treated.
