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Reduced IGF signaling prevents muscle cell death in a Caenorhabditis elegans model of muscular dystrophy
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| Zeitschriftentitel: | Proceedings of the National Academy of Sciences |
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| Personen und Körperschaften: | , |
| In: | Proceedings of the National Academy of Sciences, 110, 2013, 47, S. 19024-19029 |
| Format: | E-Article |
| Sprache: | Englisch |
| veröffentlicht: |
Proceedings of the National Academy of Sciences
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| Schlagwörter: |
| Zusammenfassung: | <jats:title>Significance</jats:title> <jats:p> Duchenne muscular dystrophy (DMD) affects 1 in 3,500 live male births and is a fatal degenerative muscle disease with no known cure. The primary cause of DMD is muscle necrosis due to the loss of the dystrophin protein in the muscle membrane. However, the underlying cellular mechanisms that lead to cell death are not known. Using <jats:italic>Caenorhabditis elegans</jats:italic> as a model of muscular dystrophy, we show that whereas loss of dystrophin function is a primary cause of muscle degeneration, muscle cell death is greatly influenced by age-dependent, intrinsically variable cellular environments. We further show that reduction of insulin-like growth factor 1 signaling, which rejuvenates the cellular environments, helps to protect against muscle cell death caused by loss of dystrophin function. </jats:p> |
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| Umfang: | 19024-19029 |
| ISSN: |
1091-6490
0027-8424 |
| DOI: | 10.1073/pnas.1308866110 |