Prions are pathologic agents that produce abnormal folding in the brain. These changes cause rapidly progressive neurological diseases that are difficult to treat and always fatal. Prions can be caused by hereditary or spontaneous mutations. They may also be acquired from an outside source, and some are transmitted from animal to human. Prion diseases are rare, affecting about 300 people every year in the U.S.
A proteinaceous infection particle or prion consists of only a protein. It does not have any genetic components that enable it to reproduce, like a virus or bacteria. Instead, prions consist of proteins present in normal cell membranes — but the protein is distorted. Researchers believe that this abnormal protein binds to proteins in healthy cells to propagate and spread the disease.
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