Category: Université de Montréal

Many researchers believe that one of the earliest events in ALS is the detachment of motor neurons from muscles at a site called the neuromuscular junction (NMJ) and have discovered that some NMJs are more vulnerable than others. In both humans and ALS laboratory mice, the eye muscles preserve the connections longer, which is why many assistive technology devices for ALS use eye movements for control. Previous work by Dr. Robitaille has revealed that specialized supporting cells called Perisynaptic Schwann Cells (PSCs) are critical for maintaining the NMJ connection and that PSC function is impaired in ALS mice. In this project, Dr. Robitaille will examine PSC function in ALS mice before and after they develop disease symptoms and compare the results to normal mice. He will also compare PSC function in eye NMJs with PSC function in leg NMJs, which are known to show early impairment. Also, Dr. Robitaille will analyze the entire set of proteins in both the resistant and susceptible NMJs and compare the results to look for specific markers that might explain the preserved function in eye muscles. If successful, this project could discover new targets for treatments that might preserve NMJs all over the body by enhancing PSC function.

Trillions of microbes including bacteria, fungi and viruses live on and in the human body in a community called the microbiota. Many of the microbes are friendly and contribute to normal, healthy functions – but others are associated with diseases. For example, changes in the collection of microbes in the respiratory tract have been linked to asthma. Recent scientific evidence has found a link between changes in the microbiota and neurodegenerative diseases like Parkinson’s disease.

To investigate whether the microbiota plays a role in ALS, Audrey Labarre, a PhD student in Dr. Alex Parker’s lab at the Université de Montréal, has been studying ALS worms. Worms are useful models for studying the biology of ALS because they have a short life span, allowing scientists to see quickly how new experimental treatments affect mobility and disease progression. When scientists alter worms by creating a genetic mutation that causes ALS, the worms develop motor neuron degeneration and paralysis. Labarre’s work to date has involved treating ALS worms with probiotics – friendly, live bacteria that are known to be beneficial to the digestive tract. She has found that certain strains of probiotics have resulted in reduced death of motor neurons as well as increased mobility in the worms. In this project, Labarre will treat ALS mice with the same probiotic strains to see if she can find similar results. This research may confirm a link between the microbiota and ALS, paving the way for future therapies that could target the bacterial population in the digestive systems of people living with ALS.