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4 result(s) found

Aberrant tRNA function exacerbates TDP-43 misfolding and toxicity in cellular models of ALS

Project Overview

Aberrant tRNA processing and function has the capacity to exacerbate misfolding of ALS-associated proteins and the ensuing toxicity. tRNAs mainly function to recruit amino acids to ribosomes, allowing faithful translation of mRNA molecules. Metabolites of processed tRNAs also function in cellular st…

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Principal Investigator:
  • Donovan McDonald, University of Western Ontario

Elucidating the role of stress granules dynamics in the pathogenesis of ALS

Project Overview

The lifetime risk of developing amyotrophic lateral sclerosis (ALS) is approximately 1 in 350 for men and 1 in 440 for women, making it the most common adult-onset motor neuron disease. There is no cure and poor prognosis for this devastating neurodegenerative disease, resulting in increased mortali…

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Principal Investigator:
  • Charlotte Manser, University of Ottawa

Investigating TDP-43 palmitoylation as a therapeutic target to lower protein aggregation

Project Overview

TDP-43 (transactive response DNA-binding protein 43) is one of the few genes highly associated with ALS (amyotrophic lateral sclerosis). Although TDP-43 mutations only occur in a small subset of ALS patients, cytoplasmic inclusions that mainly consists of TDP-43 protein aggregates are present in 97%…

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Team Members:
  • Lucia Liao, University of Waterloo

Neuronal reprogramming as a novel therapeutic strategy to treat Amyotrophic lateral sclerosis

Project Overview

The demonstration that mature somatic cells can be converted to new, heterologous cell types, now termed “cellular reprogramming”, led to the 2012 Nobel Prize in Physiology or Medicine, and sparked an explosion of studies investigating the therapeutic potential of such an approach. Direct neuron…

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Principal Investigator:
  • Hussein Ghazale, Sunnybrook Research Institute