Team 3
Biology and Physiopathology of the Spinal Cord

Early neuromuscular junction alterations and their role in motor neuron degeneration in ALS

Our research focuses on how the neuromuscular junction and muscle cells contribute to motor neuron degeneration in amyotrophic lateral sclerosis (ALS). In particular, we study early structural and molecular changes at neuromuscular junctions and aim to determine whether these alterations originate in muscle or in motor neurons.

We also investigate differences between neuromuscular junctions associated with fast and slow motor neurons to understand why fast motor neurons degenerate first. This work may help identify early biomarkers of ALS and support the development of new therapeutic strategies to preserve neuromuscular junctions and slow disease progression.

Main papers

The collagen ColQ binds to LRP4 and regulates the activation of the Muscle-Specific Kinase-LRP4 receptor complex by agrin at the neuromuscular junction

Uyen Dao, T. M., Barbeau, S., Messéant, J., Della-Gaspera, B., Bouceba, T., Semprez, F., Legay, C., & Dobbertin, A. — Journal of Biological Chemistry, 2023

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AChR β-Subunit mRNAs Are Stabilized by HuR in a Mouse Model of Congenital Myasthenic Syndrome With Acetylcholinesterase Deficiency

Karmouch, J., Delers, P., Semprez, F., Soyed, N., Areias, J., Bélanger, G., Ravel-Chapuis, A., Dobbertin, A., Jasmin, B. J., & Legay, C. — Frontiers in Molecular Neuroscience, 2020

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MuSK frizzled-like domain is critical for mammalian neuromuscular junction formation and maintenance

Messéant, J., Dobbertin, A., Girard, E., Delers, P., Manuel, M., Mangione, F., Schmitt, A., Le Denmat, D., Molgó, J., Zytnicki, D., Schaeffer, L., Legay, C., & Strochlic, L. — Journal of Neuroscience, 2015

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Other Research Topics

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Magnetic Stimulation for Spinal Cord Injury and ALS

Harnessing non-invasive magnetic stimulation to reduce inflammation and promote repair in spinal cord injury and ALS.

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Surface Physics and Mechanics of Neuronal Growth

Exploring how surface energy and cell mechanics guide axonal growth and nerve repair, from fundamental biophysics to implantable biomaterials.

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Motoneuron Excitability in ALS and Spinal Cord Injury

Investigating how ion channels and network mechanisms drive motoneuron vulnerability, bistability, and recruitment in ALS and spinal cord injury..

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