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ReportsPrion-Induced Amyloid Heart Disease with High Blood Infectivity in Transgenic MiceWe investigated extraneural manifestations in scrapie-infected transgenic mice expressing prion protein lacking the glycophosphatydylinositol membrane anchor. In the brain, blood, and heart, both abnormal protease-resistant prion protein (PrPres) and prion infectivity were readily detected by immunoblot and by inoculation into nontransgenic recipients. The titer of infectious scrapie in blood plasma exceeded 107 50% infectious doses per milliliter. The hearts of these transgenic mice contained PrPres-positive amyloid deposits that led to myocardial stiffness and cardiac disease.
1 Viral-Immunobiology Laboratory, Departments of Molecular and Integrative Neurosciences and Infectology, Scripps Research Institute, La Jolla, CA 92037, USA. * To whom correspondence should be addressed. E-mail: mbaobo{at}scripps.edu (M.B.A.O.), bchesebro{at}niaid.nih.gov (B.C.), kknowlton{at}ucsd.edu (K.U.K.)
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Science. ISSN 0036-8075 (print), 1095-9203 (online)