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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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Detalhes bibliográficos
Main Authors: Genoud, Nicolas, Behrens, Axel, Miele, Gino, Robay, Dimitri, Heppner, Frank L., Freigang, Stefan, Aguzzi, Adriano
Formato: Artigo
Idioma:Inglês
Publicado em: National Academy of Sciences 2004
Assuntos:
Acesso em linha: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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