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Regular exercise prolongs survival in a type 2 spinal muscular atrophy model mouse
Clément Grondard1, Olivier Biondi, Anne-Sophie Armand
1Université Paris Descartes, Centre Universitaire des Saints-Pères, F-75270 Paris, France.
Summary
Running exercise significantly improved motor function and lifespan in spinal muscular atrophy (SMA) mice by enhancing motor neuron survival. This neuroprotection is linked to increased SMN2 gene expression, offering potential therapeutic avenues for SMA patients.
Area of Science:
- Neuroscience
- Genetics
- Exercise Physiology
Background:
- Physical exercise demonstrates neuroprotective potential, even in neuromuscular diseases.
- Spinal muscular atrophy (SMA) is a genetic neuromuscular disorder characterized by motor neuron degeneration.
Purpose of the Study:
- To investigate the efficacy of running-based exercise in a mouse model of type 2 spinal muscular atrophy (SMA).
- To explore the underlying mechanisms of exercise-induced neuroprotection in SMA.
Main Methods:
- Utilized SMN (survival motor neuron)-null mice carrying a human SMN2 transgene as a model for type 2 SMA.
- Assessed the impact of a running-based training program on motor function, lifespan, neuronal survival, and muscle phenotype.
- Analyzed changes in alternative splicing patterns of the SMN2 gene in response to exercise.
Main Results:
- Running exercise extended the lifespan of SMA-like mice by 57.3% and sustained motor function.
- Reduced neuronal death in the spinal cord's lumbar anterior horn was observed in trained mice.
- Exercise enhanced motoneuron survival and promoted increased expression of exon 7-containing SMN2 transcripts.
Conclusions:
- Running-based exercise confers significant neuroprotection and functional benefits in a mouse model of SMA.
- The observed benefits are associated with improved SMN2 gene splicing and enhanced motoneuron survival.
- These findings suggest exercise as a potential therapeutic strategy for human SMA patients.