New York - For the first time, researchers have developed a reliable test that can be used to determine if cattle-derived products or tissues are infected with the agent that causes "mad cow disease" or bovine spongiform encephalopathy (BSE).
The test subject is a genetically altered mouse that develops neurological symptoms within 250 days after exposure to the infectious material, according to a report in the Proceedings of the National Academy of Sciences. The disease is thought to spread by infectious proteins known as prions, which contaminate neurological, lymphoid and possibly other tissue.
The transgenic mice "make possible for the first time, an accurate determination of BSE prion titers in brain and other tissues," according to senior investigator Dr. Stanley Prusiner, of the University of California, San Francisco and colleagues. Prusiner received a Nobel prize this year for his work on prions.
At least 20 young adults in the U.K. and France have developed a fatal neurological disorder, a variant of Creutzfeldt-Jakob disease, which some experts believe came from eating contaminated beef.
The mice also can be used to test cattle in countries thought to be free of BSE, including the U.S. and Canada. And the rodents can also be used to test for BSE in cattle-derived products, according to the report. Such products include collagen, which is used in plastic and reconstructive surgery and gelatin, which is used as a stabilizing agent in a variety of foods and drugs.
Many laboratory animals will develop neurological disease when exposed to BSE, but it can take years for symptoms to develop and results are inconsistent from animal to animal. The new strain of mice, known as Tg(BoPrP), carry the gene for the normal prions found in cattle, and are more likely to develop the disease once exposed to the agent.
It's thought that the infection is transmitted by abnormal prion proteins that convert the normal version into a more resistant and brain damaging molecule.
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