Prion diseases--incurable, ultimately fatal, transmissible neurodegenerative disorders of mammals--are believed to develop undetected in the brain over several years from infectious prion proteins. In a new study, National Institutes of Health (NIH) scientists report they can detect infectious prion protein in mouse brains within a week of inoculation. Equally surprising, the protein was generated outside blood vessels in a place in the brain where scientists believe drug treatment could be targeted to prevent disease. The study, from NIH's National Institute of Allergy and Infectious Diseases (NIAID), was published online in the September 22, 2015 issue of mBio. The article is titled “Early Generation of New PrPSc on Blood Vessels after Brain Microinjection of Scrapie in Mice.” The first author of the study is Bruce Chesebro, M.D., Chief of the NIAID Laboratory of Persistent Viral Diseases. Scientists believe prion diseases could potentially be treated if therapy stwere to start early in the disease cycle. However, identifying who needs treatment and pinpointing the optimal time frame for treatment are open questions for researchers. Human prion diseases include variant, familial, and sporadic Creutzfeldt-Jakob disease (CJD). The most common form, sporadic CJD, affects an estimated one in one million people annually worldwide. Other prion diseases include scrapie in sheep; chronic wasting disease in deer, elk, and moose; and bovine spongiform encephalopathy (mad cow disease) in cattle. In their study, the NIAID scientists injected infectious scrapie prion protein into the brains of mice. After 30 minutes, they began observing whether the injected material generated new infectious protein at the injection site.
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