Prion diseases occur when normal prion protein, found on the surface of many cells, becomes abnormal and clumps in the brain, causing brain damage. 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. Unfortunately, prion diseases are usually rapidly progressive and always fatal. Human prion disease include Creutzfeldt-Jakob Disease (CJD), Variant Creutzfeldt-Jakob Disease (vCJD), Gerstmann-Straussler-Scheinker Syndrome, Fatal Familial Insomnia, and Kuru.

 

 

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