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Jun 18

Research Bit: Towards understanding of neuromuscular vulnerability in ALS

Research Bits
The Packard Center welcomed Richard Robitaille from the University of Montreal to a recent Investigator's meeting.

Date: June 18, 2021

Presenter: Richard Robitaille, PhD

Talk Title: Towards understanding of neuromuscular vulnerability in ALS

What was the question being asked?

Neuronal death and loss of communication between neuron and muscle cells is a bona fide component of ALS progression. The Robitaille lab seeks to understand how these neuron – muscle connections are lost and the contribution of other central nervous system (CNS) cells to this process.

Why is this important for ALS research?

The loss of contacts and communication between neurons and muscle is a well-known phenomenon in ALS and other neuromuscular diseases. However, the mechanisms by which these connections are lost remain poorly understood and highly debated. Thus, the Robitaille lab hopes to address these gaps in knowledge to understand the precise events leading to altered neuron – muscle communication in ALS.

What was the take-home message?

Neuron – muscle contacts are dynamically remodeled prior to communication failure. The Robitaille lab has also established a role for a specific type of glial cell in the remodeling and repair of neuron – muscle contact sites. In a mouse model of ALS, the ability of this glial cell to remodel and repair neuron – muscle contacts fails.

How do you think the results of this study might impact future approaches to the treatment of ALS?

Collectively, the research in the Robitaille lab highlights the critical involvement of additional cells in maintaining proper neuron – muscle communication and defines a failure of this process as a contributor to ALS pathogenesis. As a result, this work suggests that therapeutic modulation of glial cell function may prove beneficial for prolonging neuron – muscle contacts and communication in ALS and perhaps related neurodegenerative diseases.

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