Not all proteins that you eat are good for you. In fact, some will kill you if consumed. When most think of proteins they think of a thick piece of bacon, or the powder added to mix into smoothies. Everyone knows bacteria and viruses can make us sick, but there is a third danger, and they are called Prions. Proteins are so varied that there are thousands of various kinds in your body that do different things. This means that a malformed protein, when it turns malignant can varied in the kind of damage it can cause, or even worse, stay dormant for years before beginning to show symptoms. Scientists are struggling to piece together just how prions mutate into their more dangerous forms. Their hope, is that greater understanding will help lead to a cure.
Prions are most famously known to cause Bovine Spongiform Encephalopathy, which is commonly known as Mad-Cow disease. These defective proteins attack the cells in both the spinal cord and brain, rapidly deteriorating the grey matter of the cow, and filling it with holes. Cows don’t even show symptoms of having been infected until anywhere from eight or nine months, and in some cases it can be years after infection. There had been a huge spike in bovine infections in 2006 that resulted in countless cows being slaughtered to prevent further infection.
These killer proteins can even infect humans with Creutzfeldt-Jakob disease, frequently coined as the human version of Mad Cow. The proteins are even more destructive in people infected by them. Creutzfeldt-Jakob disease is currently incurable and so far, always fatal.
Another conditions caused by prions is the fatal, Familial Insomnia Disease. This is a form of infection that can be inherited through the parent’s genes, or a mutation. During its early stages, the egg cell can trigger this disease. The infected person will first experience insomnia. The lack of sleep will negatively affect the body, bringing about a collection of secondary symptoms that aren’t directly from the prion, but are rather the result of the lack of sleep. As the prions eat away at the infected grey matter, victims will begin to experience both physical and auditory hallucinations. Finally, the last stage is coma, which leads to death after about nine months from the time symptoms began.
Work is being done to find a cure, and scientists have moved a step closer to that goal. Lab rats have been used to produce white blood cells that can seek out the chemical markers of several known prions and attack them as they would any other virus. This break through has left scientists hopeful that they might isolate these white blood cells and be able to use them to immunize humans. If successful, the hope is that no more people will died from diseases like Creutzfeldt-Jakob and Fatal Familial Insomnia.
Clearly, there are proteins that will kill you, and more will be found as more is understood about Prions; nevertheless, scientists are slowly getting the upper hand.
By Daniel O’Hare
CDC Bovine Spongiform encephalopathy