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Transmissible spongiform encephalopathies (TSEs), also known as prion diseases, [1] are a group of progressive, incurable, and fatal conditions that are associated with the prion hypothesis and affect the brain and nervous system of many animals, including humans, cattle, and sheep.
A prion / ˈ p r iː ɒ n / ⓘ is a misfolded protein that induces misfolding in normal variants of the same protein, leading to cellular death.Prions are responsible for prion diseases, known as transmissible spongiform encephalopathy (TSEs), which are fatal and transmissible neurodegenerative diseases affecting both humans and animals.
The prion gene that codes for the prion protein is highly conserved in most mammals, meaning the gene is similar and present in most species of mammals. Three locations on the prion protein gene have been identified as highly polymorphic and may have an effect on scrapie: codons 136, 154, and 171. [56]
Most forms of aseptic meningitis are viral in origin, though neoplastic and Lyme disease meningitis are also aseptic. California encephalitis virus; Central nervous system viral disease; Cytomegalovirus encephalitis; SARS-CoV-2; Eastern equine encephalitis; Enterovirus encephalitis; Epstein Barr Virus encephalitis; Herpes simplex encephalitis ...
The abnormal protein PrP Sc accumulates in the brain and destroys nerve cells, which leads to the mental and behavioral features of prion diseases. [citation needed] Several other changes in the PRNP gene (called polymorphisms) do not cause prion diseases but may affect a person's risk of developing these diseases or alter the course of the ...
Currently, no tests are available to detect signs of this illness in live animals. However, veterinary pathologists can confirm this illness by microscopic examination of the brain tissue in animals suspected to have died of this disease, where they expect to detect areas of distinct sponge-like formations, or by the identification of the prion protein in these tissue samples.
PrP systemic amyloidosis is an extremely rare and unusual form of inherited prion disease. Unlike most prion diseases, PrP systemic amyloidosis is not isolated to the central nervous system. The prion amyloid has extensive peripheral involvement, finding its way to peripheral nerves and internal organs. The initial presentation of this disease ...
Gerstmann–Sträussler–Scheinker syndrome (GSS) is an extremely rare, always fatal (due to it being caused by prions) neurodegenerative disease that affects patients from 20 to 60 years in age. It is exclusively heritable, and is found in only a few families all over the world. [ 1 ]