Prion-induced amyloid heart disease with high blood infectivity in transgenic mice |
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| Authors: | Trifilo Matthew J Yajima Toshitaka Gu Yusu Dalton Nancy Peterson Kirk L Race Richard E Meade-White Kimberly Portis John L Masliah Eliezer Knowlton Kirk U Chesebro Bruce Oldstone Michael B A |
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| Affiliation: | Viral-Immunobiology Laboratory, Departments of Molecular and Integrative Neurosciences and Infectology, Scripps Research Institute, La Jolla, CA 92037, USA. |
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| Abstract: | 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 10(7) 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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