Patients who develop prion disease do not always have exactly the same symptoms. Some have more behavioural changes, more vision problems, or balance issues. Why is this? We know that the disease is caused by an accumulation of misfolded prion protein. If this protein folds into different shapes, it might affect different areas of the brain. When a different area of brain is affected, the patient develops a different symptom. We want to know which prion shapes target which brain areas and how. Is it by spreading to that area more easily, or does a particular shape resist being removed from certain areas? To answer these questions, we grow slices of brain in a dish and infect them with prions. By infecting different regions of brain with different shapes of prions, we hope to understand the relationship between prion shape and patient symptoms. Ultimately, this will help us predict which prion shape is affecting someone, based on their symptoms, and hopefully let us choose a more targeted therapy, one designed to get rid of that particular shape of prion.
About the Researcher:
Valerie Sim, MD, FRCPC
Centre for Prions and Protein Folding Diseases, University of Alberta, Canada
Dr. Valerie Sim self-identifies as a scientist who practices medicine to support her music habit. By day she is a prion scientist at the Centre for Prions and Protein Folding Diseases at the University of Alberta and a clinical neurologist consultant for rapidly progressive dementia cases locally and throughout Canada. By night (before COVID) she is a violinist and fiddler.
After her undergraduate and medical degrees at the University of Calgary and her neurology residency at University of Ottawa, Dr. Sim completed a post-doctoral fellowship in prion disease research at Rocky Mountain Laboratories, NIH, Montana, under the supervision of Dr. Byron Caughey. She joined the University of Alberta Division of Neurology in 2009 and was promoted to associate professor with tenure in July, 2016.
In her research lab, Dr. Sim grows prion-infected brain slices in a dish in order to ask: 1) how a prion’s size and shape can influence patterns of disease and risks of transmission; and 2) how targeting multiple steps along the disease pathway might produce more effective treatments. From protein biochemistry to animal treatment experiments, her research publications have received international media attention.
Clinically, she is medical director of the Canadian CJD Association and co-founder of the Edmonton Cognitive Neurology clinic.
She is also passionate about promoting science communication and has published a TEDx talk on prion disease. She regularly presents the science of prion disease to diverse communities across Alberta, Canada, and internationally.
- The Katie Pohl Dopirak Memorial Research Grant, contributed by the Pohl and Dopirak Families
- The Jeffrey A. Smith Memorial Research Grant, contributed by The Jeffrey and Mary Smith Family Foundation; Zoë Smith Jaye and Jenny Smith Unruh; and Mary Smith
- The Robert J. Esposito Memorial Research Grant, contributed by Kathy Esposito
- The Kendall Palmer Memorial Grant, contributed by Mitch Palmer and Family
- The Strides for CJD Grant, contributed by the Families of the CJD Foundation