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Accumulation of protease-resistant prion protein (PrP) and apoptosis of cerebellar granule cells in transgenic mice expressing a PrP insertional mutation

We have generated lines of transgenic mice that express a mutant prion protein (PrP) containing 14 octapeptide repeats whose human homologue is associated with an inherited prion dementia. These mice develop a neurological illness with prominent ataxia at 65 or 240 days of age, depending on whether...

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Autori principali: Chiesa, Roberto, Drisaldi, Bettina, Quaglio, Elena, Migheli, Antonio, Piccardo, Pedro, Ghetti, Bernardino, Harris, David A.
Natura: Artigo
Lingua:Inglês
Pubblicazione: The National Academy of Sciences 2000
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Accesso online:https://ncbi.nlm.nih.gov/pmc/articles/PMC25870/
https://ncbi.nlm.nih.gov/pubmed/10805813
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