Kuru is an infectious disease, a part of a group of illnesses called TSEs or Transmissible Spongiform Encephalopathies; they are also known as ‘Prions’. Prion stands for proteinaceous infectious particle. A strange fact about prion diseases is that these infections are not caused by viruses, bacteria, or parasites but proteins. The scariest part is that these proteins cannot be destroyed, at least, not yet, but prion scientists are working on it [1-3].
Proteins are not living organisms; many are core building blocks in our bodies. So, how do proteins like prions end up causing deadly diseases?
Yes, proteins do not replicate because they have no genetic material, which makes the study of prions more fascinating. In our cells, DNA is converted to RNA, which is made into proteins. Different proteins fold in different ways — these folds are highly specialized and function as detailed instructions for many biological processes and structures. Think of them as architectural blueprints for a building.
Now, if a protein does not fold the right way, this type of misfolding can be the root cause of disorders like cystic fibrosis. Prions are misfolded proteins but the bigger problem is that become contagious. A prion acts like a bad influence or a rotten apple. It’s very proximity begins to affect neighbouring proteins and they begin to fold incorrectly. Eventually, the brains of infected individuals looked like sponges, with many pockets of missing nerve cells and no way to reverse it.
It took a few decades for scientists to understand this phenomenon because the bodies of infected people did not show any signs of infection. This disease really had the experts stumped. It wasn’t until 1970s and 1980s that the idea of infectious proteins gathered steam, leading to a Nobel Prize for this landmark discovery [3,4,11].
Thankfully, kuru is now a thing of the past but prions cause other diseases in humans like Creutzfeldt-Jakob Disease (CJD), Gerstmann-Straussler-Scheinker Syndrome, and Fatal Familial Insomnia. Mad Cow Disease in cows and other bovine animals and scrapie in sheep are some prion diseases affecting animals . So, the history, epidemiology, and further study of these mutating proteins are essential for advancing the fight against infectious diseases.