Thursday, November 3, 2016

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?
Prions are dangerous pathogens that trigger fatal brain degeneration in humans and animals. 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:

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

Corina Marinescu

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