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Early view of prion infection. (NIAID/RML)

A study at Hamilton's Rocky Mountain Laboratories (RML), recently published, shows early prion disease progression in cells of the eye. Prion diseases are fatal infections of the central nervous system in humans and other mammals. The brain is usually involved in similar diseases such as Cruetzfeldt-Jakob disease in humans and chronic wasting disease in deer and elk. Finding early prion disease development could help in finding ways to slow disease spread.

The researchers used mice infected with scrapie and looked at their eyes. With what is called confocal microscopy, they studied rods and cones in the light-detecting retina cells of the eye. They saw abnormal clusters of prion proteins start infections near structures called ribbon synapses.

The study at the Laboratory of Persistent Viral Diseases at RML found deposits of the abnormal prions next to cilia, which carry molecules between cells. In an interview with KLYQ, Researcher Jim Striebel said, "When you finally got the image from the computer and put them all together, it was really an "a-ha" moment to see this...it's kind of a horseshoe-shaped structure that's called a ribbon synapse. And right below it were these deposits of the abnormal prion protein...It suggested, just by association, that it might be interfering with the way signals travel through synapses from the eye to the brain." (see photo above)

The details of the prion protein and how it was associated with retinal damage had not been seen before. It is likely to be the same in all prion-susceptible species.

Of course, finding this step of the infection process is leading to more study at the Hamilton, Montana, NIH/NIAID facility. Bruce Chesebro, M.D., chief of the Laboratory of Persistent Viral Diseases, said there are four individual prion-centric Principal Investigators and their teams at the lab, working on separate projects for years.

He told KLYQ that they are trying to stay at the forefront of "what is possible." A key to that is certain amount of overlapping studies. Chesebro said they are pretty successful at reaching that goal "because the groups are small enough that they can talk to each other. And we have weekly meeting of all the groups in the labs." That allows sharing developments in totally different studies that might find application in the prion research.

The study was published in "Acta Neuropathologica Communications." The National Institutes of Health (NIH) includes 27 Institutes and Centers, including the National Institute of Allergy and Infectious Diseases (NIAID).

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