Prying Prions

 

We’re all made up of thousands upon thousands of cells. Each one of these cells contains proteins – insulin, histone and haemoglobin are just a few you may have heard of. Proteins are essential for repairing and making cells and play a vital role in growth, so…pretty important, right? Proteins are responsible for the majority of cellular activity, including cell shape, waste clearance and organization. Despite proteins being key to staying well, some proteins do not have positive effects on the body. In this article I will be focusing on prions: a malicious form of protein that characterize a range of neurodegenerative diseases in both humans and animals.

Prions are a form of misfolded protein, it is their ability to transmit their abnormal shape onto normal variants of the same protein that can lead to neurodegeneration. To put it simply, prions have the ability to trigger normal, healthy proteins in the brain to fold in abnormal ways. We regularly hear of bacteria and viruses causing disease, but we rarely hear of prions. Although all three are disease causing, prions differ in their mode of action as they are simply just proteins, they are devoid of the genetic material found in bacteria and viruses. To date, the way in which these prions are able to change the shape of regular proteins is still baffling scientists and research is ongoing. It is known that prion diseases can sometimes be spread to humans via infected meat products.

To truly understand the destructive nature of prions, it would be good start to look at what a neurodegenerative disease is. A neurodegenerative disease is characterised by a group of disorders that involve the progressive degeneration of the function and structure of the nervous system – they lead to the death of various areas of the brain. Some of the most common diseases in this category that you may have heard of are Alzheimer’s disease and Parkinson’s disease. Neurodegenerative diseases are extremely cruel, debilitating conditions, the majority of which are currently incurable.

Increasing evidence incriminates prions to be the cause of Alzheimer’s and Parkinson’s although this is not completely proven. An example of a neurodegenerative disease that has been identified as a “prion” disease is Creutzfeldt-Jakob Disease (CJD). Infection with this disease usually leads to death within one year of illness onset, it is rapidly progressive and sadly always fatal.  Research into the pathway of prions is essential when trying to battle such aggressive conditions as CJD. Unlike other disease-causing organisms, prions are not destroyed by radiation of extremes of heat that can be used to kill viruses and bacteria. Antibiotics and antiviral medicines have also been found to have no effect.

With continued research and increased awareness of these proteins and the disease associated with them, we can hope that one day patient prognosis will improve and we will find a way to put a stop to these abominable conditions.