Analysis of the effects of exercise in mouse models of severe human motor neuron disease.

Summary Infantile spinal muscular atrophy (ISA) and amyotrophic lateral sclerosis (ALS) are two very serious neurodegenerative diseases. We analyzed the effects of exercise in mouse models of these two diseases. Running is beneficial in the case of ASI. It increases life span, provides neuroprotection and decreases muscle atrophy due to the disease. A study comparing the effects of swimming and running in a mouse model of ALS shows that both types of exercise are neuroprotective. However, while running protects motor neurons in slow motor units, swimming protects those in fast motor units. Whether in ASI or ALS, exercise is very beneficial. These studies pave the way for the development of training programs for patients and will help identify these mechanisms and propose new therapeutic avenues.