Quick Search:

uzh logo
Browse by:

Zurich Open Repository and Archive

Maintenance: Tuesday, July the 26th 2016, 07:00-10:00

ZORA's new graphical user interface will be relaunched (For further infos watch out slideshow ZORA: Neues Look & Feel). There will be short interrupts on ZORA Service between 07:00am and 10:00 am. Please be patient.

Permanent URL to this publication: http://dx.doi.org/10.5167/uzh-1963

Flechsig, E; Hegyi, I; Leimeroth, R; Zuniga, A; Rossi, D; Cozzio, A; Schwarz, Petra; Rülicke, T; Götz, J; Aguzzi, A; Weissmann, Charles (2003). Expression of truncated PrP targeted to Purkinje cells of PrP knockout mice causes Purkinje cell death and ataxia. The EMBO Journal, 22(12):3095-3101.

View at publisher


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 Purkinje cells. The phenotype is abrogated by co-expression of full-length PrP. Doppel is 25% similar to PrP, has the same globular fold, but lacks the flexible N-terminal tail. We now show that in Zürich I-type PrP-null mice, expression of N-terminally truncated PrP targeted to Purkinje cells also leads to Purkinje cell loss and ataxia, which are reversed by PrP. Doppel and truncated PrP probably cause Purkinje cell degeneration by the same mechanism.


50 citations in Web of Science®
55 citations in Scopus®
Google Scholar™



96 downloads since deposited on 11 Feb 2008
15 downloads since 12 months

Detailed statistics

Additional indexing

Item Type:Journal Article, refereed
Communities & Collections:04 Faculty of Medicine > Psychiatric University Hospital Zurich > Division of Psychiatric Research and Clinic for Psychogeriatric Medicine
04 Faculty of Medicine > University Hospital Zurich > Institute of Neuropathology
Dewey Decimal Classification:570 Life sciences; biology
610 Medicine & health
Date:16 June 2003
Deposited On:11 Feb 2008 12:26
Last Modified:05 Apr 2016 12:21
Publisher:European Molecular Biology Organization ; Nature Publishing Group
Publisher DOI:10.1093/emboj/cdg285
Related URLs:http://www.nature.com/emboj/journal/v22/n12/abs/7590696a.html
PubMed ID:12805223

Users (please log in): suggest update or correction for this item

Repository Staff Only: item control page