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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.
All known prion diseases in mammals affect the structure of the brain or other neural tissues. These diseases are progressive, have no known effective treatment, and are invariably fatal. [9] Most prion diseases were thought to be caused by PrP until 2015 when a prion form of alpha-synuclein was linked to multiple system atrophy (MSA). [10]
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.
Also known as chronic wasting disease, "zombie deer disease" is a prion disease, a rare, progressive and fatal neurodegenerative disorder that affects deer, elk, moose and other animals, the CDC says.
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 ...
Chronic wasting disease (CWD), sometimes called zombie deer disease, is a transmissible spongiform encephalopathy (TSE) affecting deer.TSEs are a family of diseases thought to be caused by misfolded proteins called prions and include similar diseases such as BSE (mad cow disease) in cattle, Creutzfeldt–Jakob disease (CJD) in humans, and scrapie in sheep. [2]
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]
Prion diseases occur when misfolded proteins cause normal proteins to misfold themselves by mechanisms yet unknown, thereby spreading disease. Misfolded proteins can accumulate into toxic clumps ...