Article
Deletion of N-terminal residues 23-88 from prion protein (PrP) abrogates the potential to rescue PrP-deficient mice from PrP-like protein/doppel-induced Neurodegeneration.
The Journal of biological chemistry - 1 Aug 2003
Atarashi Ryuichiro, Nishida Noriyuki, Shigematsu Kazuto, Goto Shinji, Kondo Takahito, Sakaguchi Suehiro, Katamine Shigeru
Abstract excerpt
Accumulating evidence has suggested that prion protein (PrP) is neuroprotective and that a PrP-like protein/Doppel (PrPLP/Dpl) is neurotoxic. A line of PrP-deficient mice, Ngsk Prnp0/0, ectopically expressing PrPLP/Dpl in neurons, exhibits late-onset ataxia because of Purkinje cell death that is...
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