Inflammatory Pathway Leads To Duchenne Muscular Dystrophy

Main Category: Muscular Dystrophy / ALS
Article Date: 28 Mar 2007 - 18:00 PDT

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Duchenne muscular dystrophy (DMD) is characterized by progressive muscle wasting and usually results in death by the age of 30. Although mutations in the dystrophin gene cause DMD, they trigger persistent inflammation, which is probably what intensifies disease progression.

In a study that appears online in advance of publication in the April print issue of the Journal of Clinical Investigation, Denis Guttridge and colleagues from Ohio State University, Columbus, show that a signaling pathway associated with chronic inflammation (the IKK/NF-kappa-B pathway) is upregulated in both muscle cells and immune cells from individuals with DMD and from mice lacking expression of dystrophin. Impaired IKK/NF-kappa-B signaling in mice lacking expression of dystrophin (achieved by either knocking out one of the genes encoding the p65 NF-kappa-B subunit or treating the mice with an IKK inhibitor) improved their muscle function and muscle regeneration. The authors therefore suggest that the IKK/NF-kappa-B signaling pathway might provide a viable therapeutic target for the treatment of DMD.

TITLE: Interplay of IKK/NF-kappa-B signaling in macrophages and myofibers promotes muscle degeneration in Duchenne muscular dystrophy

AUTHOR CONTACT:
Denis C. Guttridge
Ohio State University College of Medicine, Columbus, Ohio, USA.

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JCI table of contents -- March 22, 2007

Contact: Karen Honey
Journal of Clinical Investigation

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