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All known prion diseases are untreatable and fatal. [9] [83] [84] Until 2015 all known mammalian prion diseases were considered to be caused by the prion protein, PrP; in 2015 multiple system atrophy was found to be transmissible and was hypothesized to be caused by a new prion, the misfolded form of a protein called alpha-synuclein. [10]
The degenerative tissue damage caused by human prion diseases (CJD, GSS, and kuru) is characterised by four features: spongiform change (the presence of many small holes), the death of neurons, astrocytosis (abnormal increase in the number of astrocytes due to the destruction of nearby neurons), and amyloid plaque formation.
After the disease had progressed into a larger epidemic, the tribal people asked Charles Pfarr, a Lutheran medical officer, to come to the area to report the disease to Australian authorities. [7] Initially, the Fore people believed the causes of kuru to be sorcery or witchcraft. [35] They also thought that the magic causing kuru was contagious.
Prion diseases, also called transmissible spongiform encephalopathies (TSEs), are neurodegenerative diseases of the brain thought to be caused by a protein that converts to an abnormal form called a prion. [16] [17] GSS is a very rare TSE, making its genetic origin nearly impossible to determine.
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 name "Creutzfeldt–Jakob disease" was introduced by Walther Spielmeyer in 1922, after the German neurologists Hans Gerhard Creutzfeldt and Alfons Maria Jakob. [7] CJD is caused by abnormal folding of a protein known as a prion. [8] Infectious prions are misfolded proteins that can cause normally folded proteins to also become misfolded. [4]
Other human prion diseases include Gerstmann-Straussler-Scheinker Syndrome and Fatal Familial Insomnia, both of which, like CJD, are extremely rare and caused by errors in the PRNP gene as well ...
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 ...