- Prions are proteins that folded improperly, and are characterized as pathogens because when they come into contact with normal proteins, they cause the normal protein to take the malformed shape of the prion, thereby spreading itself.
- Prions can appear through various ways, but the most common two are genetic disorders and consuming prions from something else that had them. The problem with the unchecked spread of out-of-shape proteins is that they eventually form into plaques and fibers that shouldn’t be there, as well as losing the functionality of the original protein. This is mostly a problem in the nervous system, the affected area of most prion diseases.
- Prion diseases are rough. All of them inevitably lead to death, and there is no cure or effective treatment for any of them yet. Currently scientists are working to find small molecule medicines that can interact with the prions, but we are a long way from seeing any effective medicine to combat prion diseases. Instead most treatment of prion diseases focuses on easing the pain and suffering of the patient until their death.
- There are only a few prion diseases in the world today, and they affect only a few thousand people every year, but there are some famous examples of prion diseases.
- The first is Mad Cow Disease(also known as Bovine Spongiform Encephalopathy), which spreads from cow to cow when they eat the meat of their brethren. It degrades the nervous system of the infected cows until they die or are slaughtered prematurely, making them seem crazy due to their erratic behavior. If a human eats the meat of an infected cow, the prions can spread to them too and cause Creutzfeldt–Jakob disease.
- Another famous and bizarre prion disease is Kuru. Native to the Fore Tribe in Papua New Guinea that eat their dead as a funereal ritual, Kuru is the Fore word for “to shake”, for the tremors that the disease causes. The disease, like most transmissible prion diseases, is caused by the consumption of someone who already had the disease. Fortunately, there are normally fewer than one case a year now.