Understanding Prion Diseases
Prion diseases are a group of rare, fatal brain diseases that occur when proteins called prions malfunction or ‘misfold’, which transform into a type of protein that can accumulate and kill brain cells. Most prion diseases take decades to become noticeable, but once active they are rapid, destructive, and fatal.
Prion diseases can occur in both humans and animals and are characterized by their long incubation periods, neurodegeneration (the loss of structure and function of neurons, often causing memory loss or forgetfulness), and a failure to induce an inflammatory response, according to the Centers for Disease Control (CDC). Known prion diseases are Creutzfeldt-Jakob Disease (CJD), Variant Creutzfeldt-Jakob Disease (vCJD), Gerstmann-Straussler-Scheinker Syndrome, Fatal Familial Insomnia, and Kuru.
CJD is the most common prion disease observed in humans, and when it manifests it can closely mimic the symptoms and warning signs of Alzheimer’s disease. While CDJ can emerge at any age, it is most commonly seen in individuals around 60 years of age. Symptoms of CDJ can include rapidly worsening confusion, Disorientation, Problems with memory, thinking, planning and judgment, and difficulty walking according to the Alzheimer’s Association. Although these symptoms can sometimes be confused with the onset of Alzheimer’s, prion diseases like CDJ are rapid, and progress at a much faster rate.
New Research on Prion Diseases
A recent study published in Proceedings of the National Academy of Sciences, conducted by researchers at the Imperial College London and the University of Zurich, has pinpointed the cause behind the transformation of normal proteins to diseased prions. These findings could prove to be monumental in the development of a cure for prion diseases, as there is currently no known curative treatment available.
When the disease manifests, the protein (PrP), attaches itself to the outer membrane of the neurons, and ‘misfolds’ into the deadly protein that is known to cause prion diseases. This protein can spread to other neuronal cells, happening rapidly and aggressively.
The study found that the ‘misfolding’ PrP was a fundamental aspect of the development of these fatal brain diseases. In their studies, scientists used a variant form of the prion protein—usually found in cases of inherited prion disease—to observe the mechanics of the disease process.
Using complex research processes such as nuclear resonance spectroscopy and computational analysis, scientists were able to uncover the ‘mechanism’ behind the causation of PrP transformation. With this, they were able to produce antibodies that could target this mechanism; something that could open doors for preventative treatments down the road.
Currently, the antibodies that have been produced are too large to pass through neuronal cells and enter the brain; however, scientists believe that their findings will be able to stop the mechanism that transforms PrP proteins and ultimately prevent the development of prion diseases. These findings could prove to be the foundation for future treatments of the disease.
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