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Holger Wille, PhD

Grant Title: Evaluating prion vaccines in transgenic mouse models of familial prion diseases

Location: University of Alberta, Edmonton, Alberta, Canada

Grant Year: 2021



We have studied the structure of the infectious prion protein, which causes Creutzfeldt-Jakob disease and other prion diseases, determining its shape and surface properties. This information allowed us to design an antigen that could mimic this structure and act as a prophylactic vaccine to prevent familial and infectious prion diseases. In the current project we plan to test our vaccine in transgenic mouse lines that carry human mutations known to cause familial prion diseases. Preliminary data from first experiments with a transgenic mouse line that models Gerstmann-Sträussler-Scheinker disease demonstrates a long-lasting protection against the effects of this particular mutation (P101L).

About the Researcher:

The general focus of the research in my laboratory is the structure of amyloids and other disease-related, misfolded proteins. In particular, we are interested in the infectious prion protein (PrPSc) and the structure-function relationship underlying its infectious nature. In recent years, mounting evidence has implicated prion-like mechanisms in other neurodegenerative diseases such as Alzheimer’s, Parkinson’s, and Lou Gehrig’s disease, to name just a few. The mechanistic similarities that connect these diseases and the archetypical prion diseases are promising research areas for future investigations. In my laboratory, the scope of our experimental approaches is centered on electron microscopy, supplemented by other biochemical and biophysical methods, and molecular modeling. Many of our experiments are carried out as interdisciplinary collaborations with other scientists from the University of Alberta and from other universities and research institutions nationally and internationally.

Recipient of:

The Strides for CJD Research Grant

Contributed by: The Families of the CJD Foundation

Funds raised by the annual Strides for CJD run/walk have been applied to research grants awarded since 2016.