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New approach to protecting prion protein from altering shape
Date:7/18/2013

ed them with Creutzfeldt-Jakob disease prions. The mice did eventually develop symptoms, but the signs did not emerge for more than a year in fact, it took about 400 days. In contrast, mice without the modified prion protein showed symptoms within 260 days.

"Our discovery that a tailor-made mutation in one specific region of prion protein can prevent it from changing shape to a disease-associated conformation helps resolve the ongoing major controversy in the field regarding the mechanism by which infectious prions self-replicate," said Qingzhong Kong, PhD, associate professor of pathology and first author on the paper.

With no effective treatments currently available for these fatal diseases, this finding has also important implications for the development of new drugs. It suggests a novel pharmacologic strategy in which scientists can either identify or design a molecule that binds to prion protein and stabilizes its normal shape, thereby preventing propagation of the disease.


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Contact: Jessica Studeny
jessica.studeny@case.edu
216-368-4692
Case Western Reserve University
Source:Eurekalert

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