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Diseases are an unavoidable consequence of living in a competitive environment like Earth. Most illnesses can be classified based on the pathogen responsible for their spread, although not all of them necessitate an external organism to transmit.
While we've grasped the nature of most diseases by now, there are still many for which we lack definitive cures. Then, there are the illnesses that defy our understanding, perplexing even the most skilled researchers. One such example is prion diseases.
Prions are unlike any other disease we've encountered, primarily because they're not technically a disease. Instead, prions are proteins found in the body that get misfolded for unknown reasons, and they can induce other proteins of the same type to misfold upon contact.
While this may seem harmless, this characteristic leads to one of the most enigmatic and fatal conditions we know of.
10. The Unsolved Mystery of Prions: Why Do They Exist (And What Are They)?
Prions are proteins found in animal tissues, primarily in the brain, spinal cord, and eyes. On their own, they aren't harmful and might even have a function in the body, though they often go rogue.
Prions have a strange tendency to alter their shape and become contagious. This means they can pass their misfolded structure to other proteins, causing them to misfold as well and spread. Depending on how the proteins misfold, it can lead to serious diseases like Creutzfeldt-Jakob disease.
Interestingly, we still don't fully understand why prions exist, since they don't seem to serve any significant purpose in the body, despite causing debilitating diseases. Some studies suggest they might play a role in nerve function.
9. They’re Incredibly Difficult to Destroy
When it comes to diseases caused by pathogens, the key is usually finding a way to eliminate or stop the external organism causing the illness. While we're still figuring out how to handle some of the more resilient species, we largely have the tools to eradicate most diseases through the traditional ‘kill ’em all’ approach.
Sadly, prions are too advanced to be destroyed using our usual methods. There have been numerous cases where people contracted prion diseases simply by coming into contact with contaminated hospital equipment. The standard disinfection methods designed to kill bacteria and viruses—such as heat and radiation—are ineffective against prions. They can reportedly endure temperatures as high as 121 degrees Celsius (249.8°F), which is more than enough to kill most known pathogens.
8. They’re Not Even Alive
Prions are particularly deadly because of their ability to quickly infect other proteins. All prion-related diseases are serious, disabling conditions. The most common is Creutzfeldt-Jakob disease (CJD), which can cause severe disability and rapid death within a year. Fortunately, most cases are seen in individuals around the age of 60, and the disease is relatively rare.
Despite the destruction they can cause, prions lack the usual characteristics of other pathogens. For one, they don't contain any genetic material like DNA or RNA, and they have no physical structures to speak of. More crucially, they aren’t even alive. Essentially, they are misfolded proteins rather than independent organisms, making them potentially more dangerous as they are not subject to the usual limitations of life and death.
7. Their Potential Use in Warfare
Biological weapons have been part of warfare for centuries, and unfortunately, their use still occurs in various global conflicts. They are alarmingly easy to acquire in certain conflict zones, making them accessible to militant groups and rogue states. Even a small-scale biological attack in a densely populated area could cause severe physical and psychological damage to its inhabitants.
Most biological weapons we know of consist of deadly strains of bacteria or viruses, but thanks to advancements in medicine and quarantine measures in many developed nations, we are improving our ability to defend against such attacks.
This is where prions become a concern. As prions aren't classified as pathogens, biological weapons involving them would be nearly undetectable and uncontrollable. Prion diseases are extremely contagious and lead to rapid neurodegenerative disorders, which could be catastrophic in the hands of, for example, a terrorist organization.
6. A Completely Different Class of Diseases
When prions were first discovered, they completely baffled scientists. It would take many years before they were even recognized as a form of disease, though due to their strange nature, we still haven't quite figured out what category of disease they fall into.
Prion diseases are unlike anything we've encountered in any branch of medicine. They are primarily caused by an accumulation of malformed proteins in organs such as the brain, leading to a range of rare and unique conditions. Many other perplexing diseases, such as Alzheimer's and Parkinson's, are now believed to be connected to prions as well.
5. We Have No Idea How Many Types Exist
Due to their rarity, prion diseases have been studied very little. While recent research has brought some understanding of these enigmatic diseases, we still don't fully grasp the mechanisms behind them. To make matters worse, we aren't even aware of all the types of prions that may exist.
Many diseases once thought to have different causes have recently been discovered to be linked to prions, as our definition of prions continues to expand. Even the types we know about are not yet fully understood, and there’s still much to learn.
4. The Link to Copper
Prion diseases are notorious for their rapid progression within the body. Typically, death occurs within months, though some cases take years. Despite extensive study, the underlying causes remain largely unknown. We still don’t understand what triggers proteins to misfold, and the rarity of prion diseases makes finding answers a formidable challenge.
However, recent findings suggest we may have uncovered a contributing factor: copper. Researchers from Iowa State University have discovered that copper ions in the body can cause prions, already present in areas like the brain and spinal cord, to misfold. While copper's connection to prions was long suspected, this is the first time it has been proven through laboratory experiments.
3. The Cure Remains Elusive
Everything we know about prion diseases makes them seem like truly devastating disorders. Most are degenerative, impairing brain function and leading to death in all cases. What’s even worse? We currently have no means of stopping the disease from claiming its victim.
Although prion diseases are rare, those who contract them must endure every stage of the illness. Some medications may help delay the symptoms, but treatment primarily focuses on providing comfort to the patient. Tragically, all prion diseases are fatal, though it may offer some relief knowing that such diseases are unlikely to affect you or anyone close to you.
2. The Time Cannibalism Spread the Disease
Cannibalism once held a certain level of acceptance in Papua New Guinea, as regular as eating in other parts of the world. The locals openly acknowledge that their ancestors practiced cannibalism, and they see no issue with it. While it's unlikely that any cannibalistic tribes still exist in Papua New Guinea, this practice continued until very recently.
Cannibalism has caused its share of health problems, which is why it's forbidden in most societies, and the people of Papua New Guinea were no exception. A striking example is the Fore tribe. When researchers visited them in the 1950s, they discovered that about 200 tribe members were dying each year from a mysterious illness. The symptoms included difficulty walking, loss of bodily control, and eventual death.
At the time, the researchers couldn’t determine the source of the illness. However, it was later discovered that prions were to blame. The disease spread through cannibalism, which was so common that even children would snack on body parts.
1. The Conspiracy Theories
Prions stand out from other diseases (or anything else we understand) in numerous ways, which is why some researchers have dubbed them the 'strangest living molecules ever'. They’re not alive, they don’t have any genetic material, and are not considered a separate species. Unsurprisingly, prions have become the subject of several conspiracy theories.
The credibility of these theories varies depending on which corner of the Internet you find yourself in, but some of them are not entirely outlandish. Certain theorists believe that governments are researching prions for their potential in experimental projects, particularly in the realm of warfare. They’ve also gained popularity among zombie apocalypse enthusiasts, as prion diseases affect the brain and could, in theory, be used to create zombies.
