Prion-induced amyloid heart disease with high blood infectivity in transgenic mice

被引：49
作者：
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. [1 ]
Chesebro, Bruce
Oldstone, Michael B. A.
机构：
[1] Univ Calif San Diego, Dept Med, La Jolla, CA 92093 USA
[2] Scripps Res Inst, Dept Mol & Integrat Neurosci, Viral Immunobiol Lab, La Jolla, CA 92037 USA
[3] Scripps Res Inst, Dept Infectol, Viral Immunobiol Lab, La Jolla, CA 92037 USA
[4] NIAID, Rocky Mt Labs, Persistent Viral Dis Lab, Hamilton, MT 59840 USA
[5] Univ Calif San Diego, Dept Neurosci, La Jolla, CA 92093 USA
[6] Univ Calif San Diego, Dept Pathol, La Jolla, CA 92093 USA
来源：
SCIENCE | 2006年 / 313卷 / 5783期
关键词：
CREUTZFELDT-JAKOB-DISEASE; TRANSMISSIBLE SPONGIFORM ENCEPHALOPATHY; DIASTOLIC DYSFUNCTION; SCRAPIE; TRANSFUSION; FAILURE; COMPONENTS; DIAGNOSIS; VARIANT; HUMANS;
D O I：
10.1126/science.1128635
中图分类号：
O [数理科学和化学]; P [天文学、地球科学]; Q [生物科学]; N [自然科学总论];
学科分类号：
07 ; 0710 ; 09 ;
摘要：
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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页码：94 / 97
页数：4
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