| Regular exercise prolongs survival in a type 2 spinal muscular atrophy model mouse. | |
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MedLine Citation:
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PMID: 16107648 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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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. |
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Authors:
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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:
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Type: Comparative Study; Journal Article; Research Support, Non-U.S. Gov't |
Journal Detail:
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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:
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Created Date: 2005-08-18 Completed Date: 2006-03-02 Revised Date: 2009-11-19 |
Medline Journal Info:
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Nlm Unique ID: 8102140 Medline TA: J Neurosci Country: United States |
Other Details:
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Languages: eng Pagination: 7615-22 Citation Subset: IM |
Affiliation:
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Université Paris Descartes, Centre Universitaire des Saints-Pères, F-75270 Paris, France. |
Export Citation:
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| MeSH Terms | |
Descriptor/Qualifier:
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Animals Cyclic AMP Response Element-Binding Protein / biosynthesis, genetics Disease Models, Animal* Female Male Mice 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 |
| Chemical | |
Reg. No./Substance:
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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 |
| Comments/Corrections | |
Erratum In:
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J Neurosci. 2005 Sep 14;25(37):8587 |
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