RML scientists presented prestigious award

Rocky Mountain Laboratories researchers Andy Hughson, Byron Caughey, Christina Orru, and Bradley Groveman were recently awarded the prestigious Aspen Institute Italia Award for their collaborative effort with a group of Italian researchers to develop a noninvasive procedure to diagnosis prion diseases.

A collaboration between scientists working in Hamilton with fellow researchers halfway around the world in Italy was recognized recently with a prestigious international award.

The 11 scientists, including four from Rocky Mountain Laboratories, will be presented with this year’s Aspen Institute Italia Award for scientific research and collaboration between the two countries next week in Italy.

“We were very pleasantly surprised and honored by the award,” said Rocky Mountain Laboratories senior investigator Byron Caughey.

RML scientists Christina D. Orru, Andrew G. Hughson and Bradley R. Groveman were members of the research team that devised a simple and non-invasive procedure for the reliable diagnosis of prion diseases, like Creutzfeldt-Jakob Disease.

Caughey said the research teams were in good company for the annual award.

“Aspen Institute considered U.S.-Italian collaborations across the breadth of natural sciences, and recent years’ awards went for studies of Ebola virus spread, black holes, and forbidden crystals,” Caughey said. “Our project seems a bit more down to earth; that is, how to analyze nasal brushings to diagnose deadly neurodegenerative diseases.”

Diagnosing neurodegenerative diseases is difficult when patients are alive because of the high risk of contamination of the brain and high costs of sterilizing health care facilities.

This research focused on developing a non-invasive test that would detect the degenerative brain disorder called Creutzfeldt-Jakob Disease (CJD).

The disease is caused when proteins called prions that normally occur in the neurons of the central nervous system begin to fold incorrectly. As the disease progresses, the mass of misfolded proteins disrupts neuronal cell function and eventually kills brain cells.

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An Italian neurologist, Dr. Gianluigi Zanusso, noticed that the olfactory linings of nasal cavities of CJD patients contained the corrupted infectious protein deposits that were known to cause the disease.

“CJD is a thankfully rare, but rapidly progressing, transmissible, untreatable, and deadly disease,” Caughey said. “Our team at RML had developed an ultrasensitive test for the corrupted CJD protein, and when Dr. Zanusso heard about it, he approached me to ask if he collected nasal brushings from CJD patients and sent them to Montana, would we test them?”

Caughey said yes and the Rocky Mountain Laboratories team of researchers found that testing the nasal specimens worked well as a diagnostic test for CJD.

The nasal brushing test takes less than a minute to collect the sample. A narrow and soft swap is inserted into a nostril and used to gently brush the top of the nasal cavity where the nerve cells that give humans their sense of smell are located. The swab is pulled out and washed into some liquid, which is then tested.

The publication “Test for Creutzfeldt-Jakob Disease Using Nasal Brushings” written by the Italian and RML research teams was the basis of the award.

“Overall interest in our approach is greatly enhanced by the possibility that a similar strategy can be applied to numerous other more common neurodegenerative diseases that are caused by the corruption of various other proteins, such as Alzheimer’s and Parkinson’s,” Caughey said. “We have since developed ultrasensitive tests for some of those other corrupted pathological proteins and are launching into the testing of nasal brushings from patients with the associated diseases.

“Ultimately, we expect that this new technology will not only improve diagnostics, but also the development of much-needed treatments for these diseases,” he said. “After all, Alzheimer’s disease alone affects about 5 million Americans and, without more effective intervention, is projected to afflict 14-15 million within about 30 years.”