$125,000 awarded to Dr. Christine Vande Velde at the University of Montréal.
The TDP-43 protein is usually found inside the cell nucleus and is responsible for regulating many cellular processes. In 97 per cent of ALS cases and nearly half of frontotemporal dementia, scientists have discovered that the TDP-43 protein is misplaced to an area outside the cell nucleus called the cytoplasm.
Dr. Vande Velde recently discovered that reducing the amount of TDP-43 protein in the nucleus caused an ALS gene called hnRNPA1 to be abnormally read, thus creating a new protein that she labeled hnRNP A1B. Dr. Vande Velde suspects this new protein could be a previously undiscovered toxic form. In her work, Dr. Vande Velde will examine how hnRNP A1B functions and whether it plays a role in known mechanisms of ALS pathology. She will conduct cell and mice experiments first and then validate her findings using ALS tissue samples generously donated to the Douglas-Bell Canada Brain Bank in Montréal and through other ALS labs.
Dr. Vande Velde hopes that a better understanding of hnRNP A1B’s function in ALS will reveal it as a potential target for new therapies and biomarkers in the future.