NR AUUO
AU Trifilo,M.J.; Yajima,T.; Gu,Y.; Dalton,N.; Peterson,K.L.; Race,R.E.; Meade-White,K.; Portis,J.L.; Masliah,E.; Knowlton,K.U.; Chesebro,B.; Oldstone,M.B.A.
TI Prion-induced amyloid heart disease with high blood infectivity in transgenic mice
QU Science 2006 Jul 7; 313(5783): 94-7
PT journal article
AB 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.
MH Amyloid/*analysis; Amyloidosis/blood/etiology/*pathology/physiopathology; Animals; Blotting, Western; Coronary Vessels/chemistry/pathology; Disease Models, Animal; Glycosylphosphatidylinositols; Heart Catheterization; Heart Diseases/blood/etiology/*pathology/physiopathology; Heart Function Tests; Immunohistochemistry; Mice; Mice, Inbred C57BL; Mice, Transgenic; Microcirculation/chemistry/pathology; Myocardial Contraction; Myocardium/*chemistry/*pathology; PrPc Proteins/chemistry; PrPsc Proteins/*analysis/blood; Research Support, N.I.H., Extramural; Scrapie/blood/*pathology/physiopathology; Staining and Labeling; Time Factors
AD Viral-Immunobiology Laboratory, Departments of Molecular and Integrative Neurosciences and Infectology, Scripps Research Institute, La Jolla, CA 92037, USA
SP englisch
PO USA