Project Overview

Amyotrophic lateral sclerosis (ALS) is a disease where motor neurons, neurons that control the muscles to generate fundamental movements in our daily life, are gradually destroyed during the disease leading to paralysis and eventually death. However, the death of motor neurons is preceded by the destruction of their contact with muscles, termed neuromuscular junction. These nerve-muscle junctions are the unique contact between neurons and muscle and are necessary for the control of muscle movements by neurons. However, despite their importance, our understanding of the changes that take place at these junctions in ALS remains quite limited. Hence, our team proposes to use advanced functional and molecular approaches to understand the changes that take place at the junctions during the disease. These approaches will allow us to analyze the functional properties and the molecular signature of the nerve-muscle junctions in various states (in contact or destroyed), thus providing us with a precise signature of the underlying mechanisms of the changes at the nerve-muscle contacts in ALS. Our team efforts will take advantage of several animal models mimicking the disease in patients, but also of model systems directly derived from patients combined with cutting-edge technologies. Our ultimate goal is to uncover mechanisms involved in the destruction of the nerve-muscle junctions during the disease, thus helping us identify potential targets that could be exploited as biomarkers and potential treatment of ALS.

Principal Investigator

Richard Robitaille , Université de Montréal

Team Members

Partners and Donors

ALS Society of Canada