Article
Immunodetection of disease-associated mutant PrP, which accelerates disease in GSS transgenic mice.
The EMBO journal - 6 Jul 2005
Nazor Karah E, Kuhn Franziska, Seward Tanya, Green Mike, Zwald Daniel, Pürro Mario, Schmid Jaqueline, Biffiger Karin, Power Aisling M, Oesch Bruno, Raeber Alex J, Telling Glenn C
Abstract excerpt
The absence of infectivity-associated, protease-resistant prion protein (PrP(Sc)) in the brains of spontaneously sick transgenic (Tg) mice overexpressing PrP linked to Gerstmann-Sträussler Scheinker syndrome, and the failure of gene-targeted mice expressing such PrP to develop disease spontaneously, challenged the concept that mutant PrP expression led to spontaneous prion production. Here, we demonstrate that...
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