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Expression of truncated PrP targeted to Purkinje cells of PrP knockout mice causes Purkinje cell death and ataxia

PrP knockout mice with disruption of only the PrP-encoding region (Zürich I-type) remain healthy, whereas mice with deletions extending upstream of the PrP-encoding exon (Nagasaki-type) suffer Purkinje cell loss and ataxia, associated with ectopic expression of Doppel in brain, particularly in Purki...

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Publicat a:EMBO J
Autors principals: Flechsig, Eckhard, Hegyi, Ivan, Leimeroth, Rainer, Zuniga, Armando, Rossi, Daniela, Cozzio, Antonio, Schwarz, Petra, Rülicke, Thomas, Götz, Jürgen, Aguzzi, Adriano, Weissmann, Charles
Format: Artigo
Idioma:Inglês
Publicat: Nature Publishing Group 2003
Matèries:
Accés en línia:https://ncbi.nlm.nih.govhttps://pmc.ncbi.nlm.nih.gov/articles/PMC162137/
https://ncbi.nlm.nih.govhttps://pubmed.ncbi.nlm.nih.gov/12805223/
https://ncbi.nlm.nih.govhttps://doi.org/10.1093/emboj/cdg285
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