A new Open Science tool for studying ALS breathing failure 

Growing respiratory motor neurons in a dish from human samples allows more targeted research

Breathing failure is a common cause of death in amyotrophic lateral sclerosis (ALS) patients. Understanding the way ALS causes breathing problems is critically important to extending their life span.  

Breathing problems mainly happen in ALS patients when their diaphragms do not respond to signals from the central nervous system. This is due to degeneration of phrenic motor neurons, a type of cell that controls the diaphragm. Studying these specific human cells would help investigate the link between ALS and breathing failure and test potential therapeutic compounds to treat respiratory symptoms. However, getting access to these cells for research can be difficult.  

Louise Thiry, a researcher in the lab of Stefano Stifani at The Neuro, has developed a way to grow these cells in a dish. Using stem cells from patient-donated samples, she generated lines of human motor neurons with varying arrangements of small molecules. She then identified which combination of molecules most successfully leads to the development of phrenic motor neurons from the stem cells.  

Taking the study a step further, Thiry then tested stem cell-derived phrenic motor neurons that held an ALS-linked gene mutation, showing that they lose electrophysiological activity and die faster and more often than those without the mutation. 

In keeping with The Neuro’s Open Science policy, Thiry has published the method to grow phrenic motor neurons open access so that scientists worldwide can use it to study ALS. The protocol might also be used to study other motor neuron diseases, including spinal muscular atrophy and Kennedy’s disease. 

“I wanted to make our work open access to speed up scientific progress by cutting barriers to collaboration and reducing inequalities in science and innovation,” says Thiry.  

To build on the work, Thiry will next compare cell survival of phrenic motor neurons with the mutation versus heathy controls in vivo. 

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The Neuro (Montreal Neurological Institute-Hospital) is a bilingual academic healthcare institution. We are a McGill research and teaching institute; delivering high-quality patient care, as part of the Neuroscience Mission of the McGill University Health Centre. We are proud to be a Killam Institution, supported by the Killam Trusts.

 

 

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