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Prions and Protein Misfolding
In very rare cases, proteins that do not fold correctly can form prions. Prions are infectious agents that can cause a variety of diseases, including mad cow disease in cattle, scrapie in sheep and Creutzfeldt-Jakob disease in humans. Prions are an aggregate of proteins that have folded incorrectly. They are transmissible and cannot be broken down by our cells and therefore usually lead to the death of the cell. In Creutzfeldt-Jakob disease, a protein found in the brain called PrP misfolds and forms PrP-sc. This incorrectly folded protein contains a high content of beta pleated sheets, which causes it to aggregate with other protein molecules to form amyloid fibers. These fibers eventually lead to the death of many neurons in the brain, leading to the degeneration of brain functionality.
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