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Normal and diseased prions. Creutzfeldt-Jakob disease and its variants belong to a broad group of human and animal diseases known as transmissible spongiform encephalopathies (TSEs). The name derives from the spongy holes, visible under a microscope, that develop in affected brain tissue.The cause of Creutzfeldt-Jakob disease and other TSEs appears to be abnormal versions of a kind of protein called a prion.ormal and diseased prions. Creutzfeldt-Jakob disease and its variants belong to a broad group of human and animal diseases known as transmissible spongiform encephalopathies (TSEs). The name derives from the spongy holes, visible under a microscope, that develop in affected brain tissue.
Prions and Prion disease. Prion diseases, also known as transmissible spongiform encephalopathies (TSE's) are a group of progressive neurodegenerative conditions. These illnesses exist in both animals and humans.Scrapie, a disease affecting sheep and goats, was the first prion disease to be identified in the 1730s.In more recent years other prion diseases have been seen in animals, the most common of which is bovine spongiform encephalopathy (BSE).ost cases of prion disease are sporadic; that is, they arise spontaneously for no known reason. More rarely prion disease is inherited due to a faulty gene, or acquired by medical procedures, transfusions, or contaminated food. Sporadic and inherited prion disease occurs worldwide in all populations.