A prion is an infectious (transmissible) protein able to replicate by transforming other proteins into a copy of the prion. The mechanism of copying is not yet understood by science.
The hypothesis was developed based on research on a nervous system disease known as Creutzfeldt-Jacob disease, which is epidemiologically associated with a bovine disease called bovine spongiform encephalitis (the mad cow disease). Research discovered that the infectious agent that causes those diseases, surprisingly, was a protein capable of copying itself and of being transmitted by ingestion (the reason why meat from contaminated animals cannot be consumed), inoculation and even heredity.
The main known human diseases caused by prions are Creutzfeldt-Jacob disease (CJD), kuru and Gerstmann-Sträussle-Scheinker disease (GSS). The hypothesis that many other diseases of unknown etiological agents are actually caused by self-replicating infectious proteins is strong.
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