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| Subject Categories: Development | Molecular Biology of Disease |
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| The EMBO Journal
(2007) 26, 481–493, doi:10.1038/sj.emboj.7601503 Published online 11 January 2007 |
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| Dissecting muscle and neuronal disorders in a Drosophila model of muscular dystrophy |
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| Halyna R Shcherbata1, Andriy S Yatsenko1, 2, Larissa Patterson1, Vanita D Sood1, Uri Nudel3, David Yaffe3, David Baker1 and Hannele Ruohola-Baker1 |
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1 Department of Biochemistry, Institute for Stem Cell and Regenerative Medicine, University of Washington, Seattle, WA, USA 2 Ivan Franko National University in Lviv, Lviv, Ukraine 3 Molecular Cell Biology, The Weizmann Institute of Science, Rehovot, Israel To whom correspondence should be addressed Hannele Ruohola-Baker, Department of Biochemistry, University of Washington, Box 357350, Seattle, WA 98195, USA. Tel.: +1 206 543 1710; Fax: +1 206 685 1792; E-mail: hannele@u.washington.edu Received 7 June 2006; Accepted 22 November 2006; Published online 11 January 2007. |
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| Abstract |
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| Perturbation in the Dystroglycan (Dg)–Dystrophin (Dys) complex results in muscular dystrophies and brain abnormalities in human. Here we report that Drosophila is an excellent genetically tractable model to study muscular dystrophies and neuronal abnormalities caused by defects in this complex. Using a fluorescence polarization assay, we show a high conservation in Dg–Dys interaction between human and Drosophila. Genetic and RNAi-induced perturbations of Dg and Dys in Drosophila cause cell polarity and muscular dystrophy phenotypes: decreased mobility, age-dependent muscle degeneration and defective photoreceptor path-finding. Dg and Dys are required in targeting glial cells and neurons for correct neuronal migration. Importantly, we now report that Dg interacts with insulin receptor and Nck/Dock SH2/SH3-adaptor molecule in photoreceptor path-finding. This is the first demonstration of a genetic interaction between Dg and InR. |
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| Keywords: axon path-finding, Dystroglycan–Dystrophin complex, insulin receptor, muscular dystrophy, Nck/Dock |
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Top of page MORE ARTICLES LIKE THISThese links to content published by NPG are automatically generated RESEARCHDissecting muscle and neuronal disorders in a Drosophila model of muscular dystrophy The EMBO Journal Article Role for α-dystrobrevin in the pathogenesis of dystrophin-dependent muscular dystrophies Nature Cell Biology Article (01 Aug 1999) Molecular Therapy Original Article β?sarcoglycan: characterization and role in limb?girdle muscular dystrophy linked to 4q12 Nature Genetics Article (01 Nov 1995) |
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