Does the unique fixed loop in the elk prion protein alter TSE pathogenesis or susceptibility?

Completed Research Project

Title / Titel: Does the unique fixed loop in the elk prion protein alter TSE pathogenesis or susceptibility?

Summary / Zusammenfassung: Chronic wasting disease (CWD) is prion disease of wild and captive deer and elk in North America which is efficiently transmitted among animals. The prevalence is startingly high in free ranging animals, up to 20% in some regions.

The structural characterization of the elk prion protein has revealed a rigid loop which has not been seen in the prion protein of other species. The loop exists at a part of the prion which has a discontinuous epitope and is suspected to be part of a protein X binding site (Kaneko et al, 1997). Using a transgenic mouse model, we will recreate this rigid loop and determine whether it may influence species barriers or whether this structural feature has an effect on the prion pathogenesis.

Project Leadership and Contacts / Projektleitung und Kontakte

Christina Sigurdson (Project Leader)	christina.sigurdson@usz.ch
Prof. Adriano Aguzzi	adriano@pathol.uzh.ch

Other Links to external Webpages: http://www.uzh.ch/pathol/neuropathologie/d/index.html

Funding Source(s) / Unterstützt durch: Others
NIH K08 Mentored Clinical Scientist Development Award

In Collaboration with / In Zusammenarbeit mit

Prof. Kurt Wüthrich, ETHZ

Switzerland