Prion prevention

Resistance and rapid testing are among potential new tools to combat prion disease.

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Transgenic mice resistant to Bovine spongiform encephalopathy (BSE) could pave the way to the breeding of resistant cattle, researchers reported this week in the Proceedings of the National Academy of Sciences (PNAS). Jiri Safar at the University of California, San Francisco (UCSF) and colleagues found that gene alterations caused mice to express a mutant form of BSE prion, which made the animals resistant when injected with high doses of the disease-causing form of prion.

BSE, also known as 'mad cow' disease, can cause variant Creutzfeldt-Jakob disease (vCJD), a rare but ultimately fatal brain disease, in humans who eat infected beef. The disease-causing agent, which is transmitted from sheep to cattle to humans, arises from a prion protein normally found on the surface of brain cells, but which has become modified and is less soluble and more resistant to enzyme degradation than the normal form.

Transgenic mice expressing resistant forms of ...

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