Article
A novel, drug-based, cellular assay for the activity of neurotoxic mutants of the prion protein.
The Journal of biological chemistry - 5 Mar 2010
Massignan Tania, Stewart Richard S, Biasini Emiliano, Solomon Isaac H, Bonetto Valentina, Chiesa Roberto, Harris David A
Abstract excerpt
In prion diseases, the infectious isoform of the prion protein (PrP(Sc)) may subvert a normal, physiological activity of the cellular isoform (PrP(C)). A deletion mutant of the prion protein (Delta105-125) that produces a neonatal lethal phenotype when expressed in transgenic mice provides a window into the normal function of PrP(C) and how it can be corrupted to produce neurotoxic effects. We report here the...
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