Prion diseases can affect both humans and animals and are sometimes transmitted to humans by infected meat products. A prion is a type of protein that can trigger normal proteins in the brain to fold abnormally. Prion diseases or transmissible spongiform encephalopathies (TSEs) are a family of rare progressive neurodegenerative disorders that affect both humans and animals. A prion disease is a type of proteopathy, or disease of structurally abnormal proteins. Creutzfeldt–Jakob disease (CJD), also known as classic Creutzfeldt–Jakob disease, is a fatal degenerative brain disorder. A prion disease (also called transmissible spongiform encephalopathy) is a disease which is caused by prions.Prions are structurally altered versions of small proteins that are normally expressed in cells. At present they cannot be treated and always end in death. Prion diseases are always degenerative, and eventually lead to death. Later symptoms include dementia, involuntary movements, blindness, weakness, and coma. The most notable genetic prion disease is Fatal Familial Insomnia, a genetic disorder present in only 30 families worldwide. A prion disease (also called transmissible spongiform encephalopathy) is a disease which is caused by prions.Prions are structurally altered versions of small proteins that are normally expressed in cells.

About 70% of people die within a year of diagnosis. 布魯希納于1982年提出了 prion 一词,以代表其自我复制能力,并能够传递本身错误结构给其它朊毒體蛋白 。 英语中的 prion 是由 pr otein and infect ion 拼接而成的 混成词 ,全称是 proteinaceous infectious particle [4] ,为「蛋白質感染颗粒」之意。 All known prion diseases in mammals affect the structure of the brain or other neural tissue. Early symptoms include memory problems, behavioral changes, poor coordination, and visual disturbances. Prions cause many forms of encephalitis, or brain disease, such as scrapie, Creutzfeldt-Jakob Disease (Bovine Spongiform Encephalopathy) and kuru. In humans, prions are believed to be the cause of Creutzfeldt–Jakob disease (CJD), its variant (vCJD), Gerstmann–Sträussler–Scheinker syndrome (GSS), fatal familial insomnia (FFI) and kuru. In animals it seems that corpses transmits prion disease and unlike in humans prions are 100% lethal. Prion diseases comprise several conditions. While normal proteins have lots of alpha helices, or twisted parts, changed proteins have lots of beta sheets, or flat parts. A prion disease (also called transmissible spongiform encephalopathy) is a disease which is caused by prions.Prions are structurally altered versions of small proteins that are normally expressed in cells. Signs and symptoms of prion disease . Prions work by changing the shape of proteins in the infected animals. They are distinguished by long incubation periods, characteristic spongiform changes associated with neuronal loss, and a failure to induce inflammatory response. Prion diseases are mostly known for affecting the brain since the prion protein-gene, a gene responsible for producing essential proteins, is especially active in the nervous system. Site-ul CDC: Prion diseases Prionii – ucigașii nevăzuți din creier , 8 iunie 2012, Mihaela Stănescu, Descoperă Primul tratament împotriva prionilor - „proteinele ucigașe” care distrug creierul , 7 aprilie 2013, Descoperă - sursa Semua penyakit ini menyerang otak atau sistem saraf lainnya, …