Dissecting muscle and neuronal disorders in a Drosophila model of muscular dystrophy

TitleDissecting muscle and neuronal disorders in a Drosophila model of muscular dystrophy
Publication TypeJournal Article
Year of Publication2007
AuthorsShcherbata, H. R., Yatsenko A. S., Patterson L., Sood V. D., Nudel U., Yaffe D., Baker D., & Ruohola-Baker H.
JournalThe EMBO journal
Date Published2007 Jan 24
KeywordsAdaptor Proteins, Signal Transducing, Animals, Animals, Genetically Modified, Cell Movement, Cell Polarity, Collaborative Publication, Disease Models, Animal, Drosophila, Dystroglycans, Dystrophin, Humans, Models, Biological, Models, Molecular, Muscular Dystrophy, Animal, Mutation, Nervous System Malformations, Oncogene Proteins

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.

Alternate JournalEMBO J.
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