Article
The toxicity of a mutant prion protein is cell-autonomous, and can be suppressed by wild-type prion protein on adjacent cells.
PloS one - 1 Jan 2012
Biasini Emiliano, Turnbaugh Jessie A, Massignan Tania, Veglianese Pietro, Forloni Gianluigi, Bonetto Valentina, Chiesa Roberto, Harris David A
Abstract excerpt
Insight into the normal function of PrP(C), and how it can be subverted to produce neurotoxic effects, is provided by PrP molecules carrying deletions encompassing the conserved central region. The most neurotoxic of these mutants, Δ105-125 (called ΔCR), produces a spontaneous neurodegenerative illness when expressed in transgenic mice, and this phenotype can be dose-dependently suppressed by co-expression of...
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