Relève étoile Jacques-Genest 
July 2018

Isabelle St-Amour

Postdoctoral fellow in Psychiatry and Neuroscience
Université Laval

Award-winning publication: Co-occurrence of mixed proteinopathies in late-stage Huntington's disease

Published in: Acta Neuropathologica


Neurodegenerative diseases such as Alzheimer's, Parkinson's and Huntingdon's are all proteinopathies: diseases in which one or several proteins - generally proteins specific to each disease - stick together, leave toxic deposits in the brains of sufferers and cause cell death in the regions that are affected. Huntingdon's, which is the focus of the research conducted by Isabelle St-Amour and her team, is no exception. The incurable genetic disease that impacts approximately 1 out of 10 000 Canadians stems from a mutation of the huntingtin protein. The investigators studied the brains of individuals who died of Huntington's disease and analyzed the normal and toxic types of proteins involved Alzheimer's and Parkinson's disease and amyotrophic lateral sclerosis. They observed an accumulation of the toxic forms of the proteins in a majority of patients with late-stage Huntington's disease. Researchers do not yet know how these toxic proteins impact the symptoms or progress of the diseases but the results should help elucidate these questions and, in the longer term, be used to develop more effective treatments for this devastating disease.