Article
The prion protein knockout mouse: a phenotype under challenge.
Prion - 1 Jan 2000
Steele Andrew D, Lindquist Susan, Aguzzi Adriano
Abstract excerpt
The key pathogenic event in prion disease involves misfolding and aggregation of the cellular prion protein (PrP). Beyond this fundamental observation, the mechanism by which PrP misfolding in neurons leads to injury and death remains enigmatic. Prion toxicity may come about by perverting the normal function of PrP. If so, understanding the normal function of PrP may help to elucidate the molecular mechansim of...
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