Article
Effects of the pathological Q212P mutation on human prion protein non-octarepeat copper-binding site.
Biochemistry - 7 Aug 2012
D'Angelo Paola, Della Longa Stefano, Arcovito Alessandro, Mancini Giordano, Zitolo Andrea, Chillemi Giovanni, Giachin Gabriele, Legname Giuseppe, Benetti Federico
Abstract excerpt
Prion diseases are a class of fatal neurodegenerative disorders characterized by brain spongiosis, synaptic degeneration, microglia and astrocytes activation, neuronal loss and altered redox control. These maladies can be sporadic, iatrogenic and genetic. The etiological agent is the prion, a misfolded form of the cellular prion protein, PrP(C). PrP(C) interacts with metal ions, in particular copper and zinc,...
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