If you fell sick, you would believe that a virus or bacteria caused it. If better educated, you would add parasites and fungi to this list. Therefore, a Prion sounded like something from Star Trek to me. Something, Captain Kirk would encounter, where no man had been before. Instead, prions are earthly creatures and can exist within you. Or in the friendly cow grazing in your backyard. When it does, the cow goes mad and its called mad cow disease.
A prion is a protein gone rogue. The protein that can turn into a prion (PrP) is plentiful in you and your cow. On deciding to turn rogue, it stops reacting to proteases. It is proteases that breaks down protein. This results in proteins misfolding. The first protein then acts like the Piped Piper and starts a chain reaction. Soon you have hordes of rogue proteins called Prion’s. Prions affect your central nervous system. They cause amyloid or plaque to form on your tissue. This disrupts the normal structure of the cell. It may take decades to surface but once it does, its actions are swift. Outcomes include brain damage and death.
Prion’s are believed to be transmitted through ingestion. When you decide to turn your cow into dinner. Do not rejoice thinking you would never eat a cow. Research has shown that plants when eaten can transmit prions. So much so a dead plant or animal even affects the soil.
Resistant to protease, heat, radiation and formaldehyde a prion is hard to kill. Current research has not solved for slowing or stopping the chain reaction. The prion follows Darwin’s theory of survival of the fittest. Once created, it uses its template to replicate prolifically. Therefore, once afflicted, the only solution is to eliminate the organism completely through culling. Or at the very least not becoming dinner.