Synthetic mammalian prions

被引：823

作者：

Legname, G

Baskakov, IV

Nguyen, HOB

Riesner, D

Cohen, FE

DeArmond, SJ

Prusiner, SB ^{[1
]}

机构：

[1] Univ Calif San Francisco, Inst Neurodegenerat Dis, San Francisco, CA 94143 USA

[2] Univ Calif San Francisco, Dept Neurol, San Francisco, CA 94143 USA

[3] Univ Calif San Francisco, Dept Mol & Cellular Pharmacol, San Francisco, CA 94143 USA

[4] Univ Calif San Francisco, Dept Biochem & Biophys, San Francisco, CA 94143 USA

[5] Univ Calif San Francisco, Dept Pathol, San Francisco, CA 94143 USA

[6] Univ Dusseldorf, Inst Phys Biol, D-40225 Dusseldorf, Germany

来源：

SCIENCE | 2004年 / 305卷 / 5684期

关键词：

D O I：

10.1126/science.1100195

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：

Recombinant mouse prion protein (recMoPrP) produced in Escherichia coli was polymerized into amyloid fibrils that represent a subset of beta sheet-rich structures. Fibrils consisting of recMoPrP(89-230) were inoculated intracerebrally into transgenic (Tg) mice expressing MoPrP(89-231). The mice developed neurologic dysfunction between 380 and 660 days after inoculation. Brain extracts showed protease-resistant PrP by Western blotting; these extracts transmitted disease to wild-type FVB mice and Tg mice overexpressing PrP, with incubation times of 150 and 90 days, respectively. Neuropathological findings suggest that a novel prion strain was created. Our results provide compelling evidence that prions are infectious proteins.

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收藏

页码：673 / 676

页数：4

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