Journal article

Alterations in Notch signalling in skeletal muscles from mdx and dko dystrophic mice and patients with Duchenne muscular dystrophy

Jarrod E Church, Jennifer Trieu, Annabel Chee, Timur Naim, Stefan M Gehrig, Severine Lamon, Corrado Angelini, Aaron P Russell, Gordon S Lynch

EXPERIMENTAL PHYSIOLOGY | WILEY | Published : 2014

Abstract

New Findings What is the central question of this study? The Notch signalling pathway plays an important role in muscle regeneration, and activation of the pathway has been shown to enhance muscle regeneration in aged mice. It is unknown whether Notch activation will have a similarly beneficial effect on muscle regeneration in the context of Duchenne muscular dystrophy (DMD). What is the main finding and its importance? Although expression of Notch signalling components is altered in both mouse models of DMD and in human DMD patients, activation of the Notch signalling pathway does not confer any functional benefit on muscles from dystrophic mice, suggesting that other signalling pathways ma..

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Grants

Awarded by National Health and Medical Research Council Australia


Funding Acknowledgements

The work was supported by the National Health and Medical Research Council Australia (project #566818). J.E.C. was supported by a postdoctoral fellowship from the Association Francaise contre les Myopathies (AFM, France). A. P. R. was supported by a National Health and Medical Research Council Biomedical Career Development Award. S. L. was supported by a Post-Doctoral Fellowship from the Swiss National Science Foundation.