Impact of prion proteins on the nerves revealed for the first time
When prion proteins mutate, they trigger mad cow and Creutzfeldt-Jakob disease. Although they are found in virtually every organism, the function of these proteins remained unclear. Researchers from the University of Zurich and the University Hospital Zurich now demonstrate that prion proteins, coupled with a particular receptor, are responsible for nerve health. The discovery could yield novel treatments for chronic nerve diseases.
What are prions?
Although they start out as harmless brain proteins, when prions become misfolded, they turn into contagious pathogens that recruit any other prions they come into contact with, grouping together in clumps that damage other cells and eventually cause the brain itself to break down.
If that's not bad enough, mutated prions can't easily be killed by heat or radiation, meaning once they've come into contact with something like surgical tools, they can potentially spread to other patients.
In the 1990s, they were responsible for the BSE epidemic more commonly known as mad cow disease. In humans, they cause Creutzfeldt-Jakob disease and other neurological disorders that are fatal and untreatable.
Meanwhile, we know that infectious prions consist of a defectively folded form of a normal prion protein called PrPC located in the neuron membrane. The infectious prions multiply by kidnapping PrPC and converting it into other infectious prions.
But what are the repercussions for the organism if the prion protein is deactivated?
Absent prion proteins cause nerve diseases
This discovery solves a key question that has long puzzled neuroscientists and points towards future applications in hospitals.
Source & further reading:
http://www.media.uzh.ch/en/Press-Releases/2016/Prion-Proteine.html
Image: Without the prion proteins, the so-called Schwann cells around the sensitive nerve fibers no longer form an insulating layer to protect the nerves.
Credit: NatureReview / Neuroscience
#neuroscience #prionproteins #humanbrain #medicine #health
Corina Marinescu You write 'What are prions? Prions are dangerous pathogens.' But it's only the degenerate form (exactly a abnormal shape of the prion) of prions that is dangerous, you precise this further in your commentary: 'we know that infectious prions consist of a defectively folded form of a normal prion protein'.
ReplyDeleteIn my opinion the issue is that those who are not aware of the prions proteins system, may still believe that all prions are dangerous, like at the time when the prions are discovered as the cause of the BSE disease. But later, scientists discovered that prions exist normally in the organism and that they have a siginificative role,: you mention rightly: 'Absent prion proteins cause nerve diseases'.
Bertrand Nelson actually she didn't write that, she provided an excerpt from the source article in which that was written. But as you say, the author went on to clarify.
ReplyDeleteBertrand Nelson Whatever the category, microbes, prions, ambiguities, genes even, the subcategory of the pathogenic steals attention. The others are too elusive to notice until big means are put to sort out their real influence.
ReplyDeleteIf you're not familiar with neuro or other medical term...well there's a bit of ambiguity, but that is the case for most of the things we do not know. So, I'll try to change it a bit...
ReplyDeleteI just put myself in the place of a reader without much prior knowledge of the problem. I personally fully understand the intricacies of your post. I notice your changes in the text Corina Marinescu.
ReplyDeleteGood night.