Permanent URL to this publication: http://dx.doi.org/10.5167/uzh-1911
Rossi, D; Cozzio, A; Flechsig, E; Klein, M A; Rülicke, T; Aguzzi, A; Weissmann, C (2001). Onset of ataxia and Purkinje cell loss in PrP null mice inversely correlated with Dpl level in brain. The EMBO Journal, 20(4):694-702.
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PrP knockout mice in which only the open reading frame was disrupted ('Zürich I') remained healthy. However, more extensive deletions resulted in ataxia, Purkinje cell loss and ectopic expression in brain of Doppel (Dpl), encoded by the downstream gene, PRND: A new PrP knockout line, 'Zürich II', with a 2.9 kb PRNP: deletion, developed this phenotype at approximately 10 months (50% morbidity). A single PRNP: allele abolished the syndrome. Compound Zürich I/Zürich II heterozygotes had half the Dpl of Zürich II mice and developed symptoms 6 months later. Zürich II mice transgenic for a PRND:-containing cosmid expressed Dpl at twice the level and became ataxic approximately 5 months earlier. Thus, Dpl levels in brain and onset of the ataxic syndrome are inversely correlated.
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|Item Type:||Journal Article, refereed|
|Communities & Collections:||04 Faculty of Medicine > University Hospital Zurich > Institute of Neuropathology|
|DDC:||570 Life sciences; biology
610 Medicine & health
|Date:||15 February 2001|
|Deposited On:||11 Feb 2008 12:26|
|Last Modified:||29 Dec 2013 09:06|
|Publisher:||European Molecular Biology Organization ; Nature Publishing Group|
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