Article
Functionally relevant domains of the prion protein identified in vivo.
PloS one - 7 Sept 2009
Baumann Frank, Pahnke Jens, Radovanovic Ivan, Rülicke Thomas, Bremer Juliane, Tolnay Markus, Aguzzi Adriano
Abstract excerpt
The prion consists essentially of PrP(Sc), a misfolded and aggregated conformer of the cellular protein PrP(C). Whereas PrP(C) deficient mice are clinically healthy, expression of PrP(C) variants lacking its central domain (PrP(DeltaCD)), or of the PrP-related protein Dpl, induces lethal neurodegenerative syndromes which are repressed by full-length PrP. Here we tested the structural basis of these syndromes by...
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