URBANA — Diseases like Mad Cow Disease and Chronic Wasting Disease among deer have generated numerous headlines over the past decade. They also share something else in common-their origin. That is what a team of researchers from the University of Illinois and Illinois Department of Natural Resources is tackling through what is likely to be groundbreaking research.

"Prions are proteins that occur in the brain of every animal," explained Jan Novakofski, a professor in the U of I Department of Animal Sciences and one of three lead researchers on the project. "In rare cases, these prions change into something that causes brain cells to die, a poison if you will. Once the process — prion disease — starts, it cannot be stopped and to date no one has found a way to inactivate these agents. Prion disease in animals and humans is terminal."

Several years ago, the British beef industry was devastated by Mad Cow Disease (BSE), a prion malady. A number of humans fell victim to the disease after eating beef infected with Mad Cow Disease and contracting the human version, Creutzfeldt-Jakob Disease (CJD). This fall, Chronic Wasting Disease began showing up in deer populations in Illinois, likely migrating from Wisconsin where the disease has had a major impact on the hunting and tourism industry.

"There are many unknowns about these prion diseases," said John Killefer, an associate professor of muscle biology in the Department of Animal Sciences and also a lead researcher on the project. "No one understands where the animals are picking up the disease and how they are passing it on."

Nohra Mateus-Pinilla, an assistant professional scientist / epidemiologist with the Illinois Department of Natural Resources' Illinois Natural History Survey and also a leader in the prion project, says it is important to establish what prompts the prions to turn bad in the first place and how the process becomes infectious.

"All three of us were interested in prion diseases so we decided to put our expertise together and develop a multi-disciplinary research approach that would benefit the livestock and agricultural industry as well as conservation and the protection of our natural resources," she explained. "Our work is partly focused on Chronic Wasting Disease but we are also looking at natural resistance to other prion diseases like scrapie in sheep and Mad Cow Disease and its human form, CJD."

The project also involves researchers from the U of I College of Veterinary Medicine and other state and federal agencies.

"Our long term goal is to understand mechanisms of CWD transmission and highlight possible intervention and prevent strategies," Mateus-Pinilla said.

Four broad goals are driving the research, the three explained. The first is the generation of a "risk map" that will aid DNR in preventive management of CWD. Based on genetic data first developed at the Rocky Mountain Laboratory in Colorado, the researchers' map will indicate areas in Illinois where deer have a "genetic susceptibility" to CWD.

"This map will overlap ecosystems favored by deer and deer densities in urban and rural Illinois, as well as CWD-positive and negative deer, land cover data, and Illinois deer genetic fingerprinting," said Mateus-Pinilla. "This information will help the development of science-based deer herd management using spatial dynamics of CWD in a host-environment framework."

A second goal involves determining if there are environmental reservoirs that favor development and transmission of the disease.

"Scientific evidence suggests that prions may persist in infectious environmental reservoirs outside deer," said Killefer. "These reservoirs may include soil, vegetation, insects or other animals. If we can determine where these reservoirs are, such knowledge may provide critical control points for intervention and prevention."

Scientific evidence has already demonstrated the ability of infectious prions to jump species. At the same time, the researchers will address the transmission dynamics of interspecies prion infection.

"The potential for interspecies transmission highlights the need to assess the potential transmission of prion diseases from wild species into livestock species-and vice versa-as well as their transmission to humans. The first step is therefore to understand mechanisms of disease transmission," said Mateus-Pinilla.

The first three goals will help lay the ground work for the fourth-the development of prevention strategies.

"Studies in sheep have demonstrated that certain genetic backgrounds are highly resistant to scrapie infection," said Novakofski. "This type of genetic resistance has not been identified in cattle or pigs. At the same time, mouse model systems have shown that gene therapy can, in effect, knock the prion protein out.

"We'll look at the potential of developing a genetic prion 'knockout' in cattle that will be naturally resistant to infection and unable to transmit the disease to other animals or humans."

As the United States has remained relatively isolated from prion disease problems until the spread of Chronic Wasting Disease from western deer populations into the Midwest, there has been little funding. The three scientists see that as changing.

One reason is the potential economic impact on the Midwestern as well as the U.S. livestock industry if CWD jumps species or if Mad Cow Disease breaks out in the United States. The standard approach-and currently the only one-is to kill the infected animals and quarantine the area. In Canada, some elk breeders whose animals were found to have CWD have not only been banned from livestock production but also prohibited from growing crops on their land.

Killefer noted that although they can be infected by artificial means, no one has been able to demonstrate the natural occurrence of prion diseases in swine. Any prion outbreak among Illinois livestock could have serious economic repercussions for the state's agribusiness sector.

"Mad Cow Disease, although controlled in Great Britain and yet to reach the United States, continues to spread in Europe and Asia," said Novakofski. "It seems prudent to redouble our efforts to deal with the prion diseases."