Article
Prion protein with an octapeptide insertion has impaired neuroprotective activity in transgenic mice.
The EMBO journal - 6 Jun 2007
Li Aimin, Piccardo Pedro, Barmada Sami J, Ghetti Bernardino, Harris David A
Abstract excerpt
Familial prion diseases are due to dominantly inherited, germline mutations in the PRNP gene that encodes the prion protein (PrP). The cellular mechanism underlying the pathogenic effect of these mutations remains uncertain. To investigate whether pathogenic mutations impair a normal, physiological activity of PrP, we have crossed Tg(PG14) mice, which express PrP with an octapeptide insertion associated with an...
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