$75,000 awarded to Maria José Castellanos Montiel, a PhD student in Dr. Thomas Durcan’s lab at the Montreal Neurological Institute and Hospital, McGill University.
The neuromuscular junction (NMJ) is the place where motor neurons, the nerve cells that control voluntary muscles, connect to muscle fibers. This junction allows for signals from the brain to pass to muscles in order to perform voluntary movement. Many researchers believe that one of the earliest events in ALS is the detachment of motor neurons from muscles at the NMJ. Thus, it is important to develop models in the laboratory to study the NMJ, to better understand how it may be impaired in ALS.
With this award, Maria proposes to create a 3D model of the NMJ using induced pluripotent stem cells (iPSCs). iPSCs have become an invaluable tool when studying neurodegenerative disease as these cells retain the genetic information of the patient who donated them and can be transformed into motor neurons, or any other cell type in the lab.
With iPSCs derived from patients who carry different ALS-linked mutations, Maria plans to create 3D NMJ models with different genetic backgrounds to investigate how the NMJ and muscle function may be affected. The ultimate goal of this project is to develop models that can used for drug screening in order to identify compounds that may have a positive effect on NMJ function and someday have the potential to be advanced into promising clinical trials.