Prion protein is a special time of protein that works in our body and is generally present in neurons in our brains. Proteins compose most of our body and are made from amino acids.
Prion is a misfolded version (wrong secondary and tertiary structure) of prion protein that is transformed to an infectious particle that leads to disease. How does prion come about? Generally, when proteins get misfolded in our body, they are either sent to be repaired or get degraded by protease (an enzyme that breaks down proteins). Prions, however, are resistant to the protease and remain misfolded and undegraded. These prions can transform other normal prion proteins to become abnormal. The misfloding in the prion is due to it's higher concentration of beta sheets (about 43% as compared to the 3% from the normal prion protein).
Prion diseases can affect both humans and animals, and are sometimes spread to humans from eating animal meat. The most common form of prion disease that affects humans is Creutzfeldt-Jakob disease (CJD). What happens with the misfolded prions? They accumulate and clump up in the brain leading to grave consequences such as personality changes, memory loss, and difficulties with movement. Unfortunately, prion diseases are fatal.
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