Journal article

Expression profiling in exercised mdx suggests a role for extracellular proteins in the dystrophic muscle immune response

Chantal A Coles, Lavinia Gordon, Liam C Hunt, Tracie Webster, Adam T Piers, Christopher Kintakas, Keryn Woodman, Su L Touslon, Gayle M Smythe, Jason D White, Shireen R Lamande

Human Molecular Genetics | OXFORD UNIV PRESS | Published : 2020


Duchenne muscular dystrophy (DMD) is a lethal muscle wasting disorder caused by mutations in the DMD gene that leads to the absence or severe reduction of dystrophin protein in muscle. The mdx mouse, also dystrophin deficient, is the model most widely used to study the pathology and test potential therapies, but the phenotype is milder than human DMD. This limits the magnitude and range of histological damage parameters and molecular changes that can be measured in pre-clinical drug testing. We used 3 weeks of voluntary wheel running to exacerbate the mdx phenotype. In mdx mice, voluntary exercise increased the amount of damaged necrotic tissue and macrophage infiltration. Global gene expres..

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Awarded by National Health and Medical Research Council of Australia

Funding Acknowledgements

This work was supported by Muscular Dystrophy Australia, the Murdoch Children's Research Institute, the National Health and Medical Research Council of Australia (1043837 to S.R.L., 1066430 to S.R.L. and J.D.W.) and the Victorian Government's Operational Infrastructure Support Program.