New Studies to Focus on Prion Diseases

CWD is one form of a group of fatal brain diseases called transmissible spongiform encephalopathies (TSEs). These diseases include bovine spongiform encephalopathy (BSE or mad cow disease) in cattle, scrapie in sheep, and Creutzfeldt-Jakob disease in humans. The hallmark of TSE disease is accumulation in the brain of abnormal prion proteins. Scientists do not know yet whether deer or elk with CWD might also transmit
some form of TSE disease to people who eat or have close contact with them.

"We must determine whether CWD is a threat to our food supply and how best to stop the spread of this disease in our deer and elk herds,” said HSS Secretary Tommy G. Thompson. "We will aggressively pursue innovative methods to expand research and direct assistance to states to fight the spread of CWD.”

Researchers will investigate the mechanics of CWD infection in deer and elk, especially in the immune system's lymphoid tissues, to aid the search for improved diagnostics and therapies. The researchers also will seek to better understand the entire spectrum of disease transmission and under what circumstances CWD might "jump” to other species. In addition, scientists will work on a possible vaccine to prevent the spread of CWD in deer and elk.

Scientists at the Rocky Mountain Laboratory (RML, Hamilton, Montana) of the US National Institute of Allergy and Infectious Diseases plan to investigate whether the abnormal CWD prion protein, presumed to be the agent that causes the disease, can be transmitted from deer or elk to monkeys if the monkeys eat meat containing the abnormal prion proteins. That knowledge would provide valuable insight into whether or not CWD could be transmitted to humans. Recently, RML scientists established that another TSE disease, hamster scrapie, could jump species--adapting to and causing disease in mice.

RML scientists also are collaborating with other researchers to test several new compounds, developed by NIAID scientists, that show promise in blocking the formation of abnormal prion proteins and therefore may one day be used as therapies to treat CWD or other TSE diseases. Some of these compounds may be able to decontaminate meat or other products infected with abnormal prion proteins.





Related Links:
US Department of Health and Human Services