The toxicity of a mutant prion protein is cell-autonomous, and can be suppressed by wild-type prion protein on adjacent cells.

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 illn...

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Bibliographic Details
Main Authors: Emiliano Biasini, Jessie A Turnbaugh, Tania Massignan, Pietro Veglianese, Gianluigi Forloni, Valentina Bonetto, Roberto Chiesa, David A Harris
Format: Article
Language:English
Published: Public Library of Science (PLoS) 2012-01-01
Series:PLoS ONE
Online Access:http://europepmc.org/articles/PMC3299791?pdf=render