• Biochemistry

    What is Prion?

    Prion, an abnormal form of a normally harmless protein found in the brain that is responsible for a variety of fatal neurodegenerative diseases of animals, including humans, called transmissible spongiform encephalopathies. In the early 1980s American neurologist, Stanley B. Prusiner and colleagues identified the “proteinaceous infectious particle,” a name that was shortened to “prion” (pronounced “pree-on”). Prions can enter the brain through infection, or they can arise from mutations in the gene that encodes the protein. Once present in the brain prions multiplies by inducing benign proteins to refold into the abnormal shape. This mechanism is not fully understood, but another protein normally found in the body may also be…

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