It can be a scary thing to think about something that it supposed to be good for your body turning around and harming it. This is exactly the case with proteins called prions.
In a village in Papua New Guinea, a strange disease had been spreading for years killing women and children in the tribe- its source and cause was elusive. When researchers delved into the matter in the early 1960s, the answer they got surprised them.
The disease was known as kuru among the people, meaning “shivering” or “trembling”. It would begin to take over an individual by slowly causing the inability to move limbs, then the loss of control of emotions, followed by the loss of control of bodily functions and complete inability to move. The village was losing about 200 people a year to the disease- mostly women and children.
After conducting extensive research in the village, researcher Shirley Lindenbaum found that it was not correlated with genetics, but with an unnerving funeral ritual. Some of the people of the tribe, mostly only women, would cook and ingest the bodies of deceased tribe members. They thought it to be an act of love, and believed they were giving the spirit of the deceased a safe place of housing for a time. Children would sometimes partake, thus the link was connected to those dying of the disease. However, it was found not be a bacteria or infection that caused the disease, but proteins gone bad.
A prion is a protein inside our brains that, when becoming twisted in the wrong way, or “misfolded”, turns hostile and combative, acting like a pathogen and causing any other protein it comes into contact with to also become warped. This forms clumps of prions that will attack brain cells until the brain completely loses functionality.
These proteins turned to the dark side are the cause of “mad cow” disease, among others, and have been linked to Alzheimer’s and Parkinson’s. It is disturbing that these proteins intended for good have the potential to harm so immensely. Yet more disturbing still is that the “infected” prions are not killed by boiling water, or easily with heat or radiation. This means the warped prions can carry over to other patients from contact with surgical tools, and the reason why mad cow disease is spread through the ingesting of affected meat. Since thorough cooking does not get rid of bad prions, one is not be able to know they are infected until later. Likewise, with the case of kuru, the disease began to spread due to one of the deceased tribal members already having a prion-related disease.
Prion-related diseases, known as transmissible spongiform encephalopathies or TSEs, are untreatable since not much information is yet known how and why prions become misfolded. Researches at Iowa State University have linked copper ions with the misfolding of the proteins, but not with the actual prion-related disease. However, the link is a start, and for now, scientists are hard at work to determine the root cause so that when symptoms of TSEs arise, the misfolding of the prions can be undone.