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Disruption of Doppel prevents neurodegeneration in mice with extensive Prnp deletions

The Prnp gene encodes the cellular prion protein PrP(C). Removal of its ORF does not result in pathological phenotypes, but deletions extending into the upstream intron result in cerebellar degeneration, possibly because of ectopic cis-activation of the Prnd locus that encodes the PrP(C) homologue D...

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Autori principali: Genoud, Nicolas, Behrens, Axel, Miele, Gino, Robay, Dimitri, Heppner, Frank L., Freigang, Stefan, Aguzzi, Adriano
Natura: Artigo
Lingua:Inglês
Pubblicazione: National Academy of Sciences 2004
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Accesso online:https://ncbi.nlm.nih.gov/pmc/articles/PMC384718/
https://ncbi.nlm.nih.gov/pubmed/15007175
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1073/pnas.0400131101
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