Prion
Pathogenic type of misfolded protein
Prion
A prion ( ) is a misfolded protein that induces folding problems in normal variants of the same protein, leading to cellular death. Prions are responsible for prion diseases, which are fatal and transmissible neurodegenerative diseases affecting animals, including humans. These proteins can misfold sporadically, due to genetic mutations, or through exposure to an already misfolded protein, leading to an abnormal three-dimensional structure that can propagate misfolding in other proteins. The term prion derives from "proteinaceous infectious particle". Unlike other infectious agents such as viruses, bacteria, and fungi, prions do not contain nucleic acids (DNA or RNA). Prions are primarily twisted isoforms of the major prion protein (PrP), a naturally occurring protein with an uncertain function. They are the hypothesized cause of various diseases, including scrapie in sheep, chronic wasting disease (CWD) in deer, bovine spongiform encephalopathy (BSE) in cattle (mad cow disease), and Creutzfeldt...
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