Journal
SCIENCE
Volume 313, Issue 5783, Pages 94-97Publisher
AMER ASSOC ADVANCEMENT SCIENCE
DOI: 10.1126/science.1128635
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Funding
- NHLBI NIH HHS [5R01HL66424-04] Funding Source: Medline
- NIA NIH HHS [P01 AG004342] Funding Source: Medline
- NINDS NIH HHS [NS041219-05] Funding Source: Medline
- PHS HHS [AGO4342] Funding Source: Medline
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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 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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