Prion-Induced Amyloid Heart Disease with High Blood Infectivity in Transgenic Mice

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Science  07 Jul 2006:
Vol. 313, Issue 5783, pp. 94-97
DOI: 10.1126/science.1128635

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We 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.

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