Document Detail

Regular exercise prolongs survival in a type 2 spinal muscular atrophy model mouse.
MedLine Citation:
PMID:  16107648     Owner:  NLM     Status:  MEDLINE    
Several studies indicate that physical exercise is likely to be neuroprotective, even in the case of neuromuscular disease. In the present work, we evaluated the efficiency of running-based training on type 2 spinal muscular atrophy (SMA)-like mice. The model used in this study is an SMN (survival motor neuron)-null mouse carrying one copy of a transgene of human SMN2. The running-induced benefits sustained the motor function and the life span of the type 2 SMA-like mice by 57.3%. We showed that the extent of neuronal death is reduced in the lumbar anterior horn of the spinal cord of running-trained mice in comparison with untrained animals. Notably, exercise enhanced motoneuron survival. We showed that the running-mediated neuroprotection is related to a change of the alternative splicing pattern of exon 7 in the SMN2 gene, leading to increased amounts of exon 7-containing transcripts in the spinal cord of trained mice. In addition, analysis at the level of two muscles from the calf, the slow-twitch soleus and the fast-twitch plantaris, showed an overall conserved muscle phenotype in running-trained animals. These data provide the first evidence for the beneficial effect of exercise in SMA and might lead to important therapeutic developments for human SMA patients.
Clément Grondard; Olivier Biondi; Anne-Sophie Armand; Sylvie Lécolle; Bruno Della Gaspera; Claude Pariset; Hung Li; Claude-Louis Gallien; Pierre-Paul Vidal; Christophe Chanoine; Frédéric Charbonnier
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Publication Detail:
Type:  Comparative Study; Journal Article; Research Support, Non-U.S. Gov't    
Journal Detail:
Title:  The Journal of neuroscience : the official journal of the Society for Neuroscience     Volume:  25     ISSN:  1529-2401     ISO Abbreviation:  J. Neurosci.     Publication Date:  2005 Aug 
Date Detail:
Created Date:  2005-08-18     Completed Date:  2006-03-02     Revised Date:  2009-11-19    
Medline Journal Info:
Nlm Unique ID:  8102140     Medline TA:  J Neurosci     Country:  United States    
Other Details:
Languages:  eng     Pagination:  7615-22     Citation Subset:  IM    
Université Paris Descartes, Centre Universitaire des Saints-Pères, F-75270 Paris, France.
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MeSH Terms
Cyclic AMP Response Element-Binding Protein / biosynthesis,  genetics
Disease Models, Animal*
Mice, Knockout
Mice, Transgenic
Motor Neurons / pathology,  physiology
Nerve Tissue Proteins / biosynthesis,  genetics
Physical Conditioning, Animal / methods*
RNA-Binding Proteins / biosynthesis,  genetics
SMN Complex Proteins
Spinal Muscular Atrophies of Childhood / genetics*,  mortality*,  pathology
Survival Rate
Survival of Motor Neuron 2 Protein
Time Factors
Reg. No./Substance:
0/Cyclic AMP Response Element-Binding Protein; 0/Nerve Tissue Proteins; 0/RNA-Binding Proteins; 0/SMN Complex Proteins; 0/SMN2 protein, human; 0/Survival of Motor Neuron 2 Protein
Erratum In:
J Neurosci. 2005 Sep 14;25(37):8587

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