Prions are infectious proteins within cells that cause normal proteins to misfold and clump together. Prions can occur as a result of a mutation or ingestion, e.g., Mad Cow disease (eating infectious beef from an animal with the disease). Creutzfeldt-Jakob disease and scrapie are prion diseases in both animals and humans. The diseases are progressive, have no cure, and end in death. Using your chemistry knowledge, explain the devastating effects of these diseases.

What will be an ideal response?


Prions are proteins that cause other cellular proteins to misfold and clump, producing a chain reaction resulting in the structural change of proteins. When the structure of a protein changes, the function of that protein changes. So as a result of infectious prion proteins, normal cellular proteins cease to exist in their normal state and, therefore, do not function in their normal way. Because proteins are so prevalent inside of cells and have so many functions that are essential, including enzymatic actions, the cell cannot function normally and dies.

Anatomy & Physiology

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