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Montana lab research honored for work on on a bizarre family of brain-wasting diseases
A leading scientific journal has lauded the research of Montana scientists on a bizarre family of brain-wasting diseases as among the top five discoveries of infectious diseases of the past decade.
By JENNIFER McKEE
Gazette State Bureau
The journal Nature Medicine ranked a 1994 discovery by scientists at Rocky Mountain Laboratories in Hamilton as a "major biomedical achievement" in its November edition.
Rocky Mountain Labs scientists showed that a normal brain protein will change its shape when in the presence of other, infectious malformed proteins culled from the brains of animals suffering from brain-wasting disease. The discovery was significant because such misshapen brain proteins are at the root of the family of brain-wasting diseases that include chronic wasting disease in deer and elk, mad cow disease in cattle and a similar lethal brain infection in people.
The diseases are thought to have a radical source, said Byron Caughey, a Rocky Mountain Labs scientist and one of the co-authors of the paper. Every other infectious disease known to science is caused by something – either a bacteria or a virus – that has its own genetic material and can replicate itself the every cell in the human body does. But this family of brain wasters seems to be caused by an inert substance – a common protein that for some reason misfolds itself, clumps up in the brain and causes deadly holes to form in brain tissue.
These diseases are always fatal and can jump from species to species, the way people got brain-wasting disease from eating the meat of infected cows in Europe in the mid-1990s.
But until 1994, Caughey said, no one had ever shown that such misshapen proteins, called prions, could actually infect other normal proteins.
"We thought it might," he said. "We hoped it might. That’s why we did the experiment."
Caughey compared the strange infectious nature of prions to the way mineral crystals will form around other crystals. Crystals aren’t infectious the way a cold is infectious. But one crystal "seeded" in a solution will cause other crystals to form.
The 1994 experiment paved the way for a host of other discoveries, he said, and the picture of how prions work in the brain is much clearer than it was 10 years ago.
Still, there’s a lot science doesn’t know. For example, the Rocky Mountain Labs’ experiment showed that normal brain proteins will convert to abnormal, disease-causing prions, Caughey said. But it didn’t show that the new prions are themselves infectious.
"That’s still the missing link," he said.
It’s possible that something else associated with prions – something science hasn’t yet discovered – is actually what causes such brain wasters.
Caughey and co-author David Kocisko, also at Rocky Mountain Labs, said that while they were proud of the 1994 discovery, they never expected it to be ranked among the top five discoveries of infectious diseases.
"I was kind of surprised," Kocisko said, who was 23 and working on his doctorate when the paper was published.
"I’m humbled."
Rocky Mountain Labs is a branch of the National Institute for Allergies and Infectious Diseases, an arm of the federal National Institutes of Health.
Copyright © The Billings Gazette, a division of Lee Enterprises.
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