Prions are miss-folded proteins. When your body makes proteins they start off as a long chain of amino acids. These amino acids bend and interact with each other to give the protein its 3D shape as well as its function. Prions are proteins which have folded in such a way that they encourage further miss folding of the same protein.
This miss folding ultimately results in lack of function and the cell hosting them to die and eventually the host. There is no known cure and the disease rapidly progresses.
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