Humanizing the mdx mouse model of DMD: the long and the short of it | DuchenneXchange

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Scientific Articles

Humanizing the mdx mouse model of DMD: the long and the short of it

key information

source: NPJ Regenerative medicine

year: 2018

authors: Nora Yucel, Alex C. Chang, John W. Day, Nadia Rosenthal, Helen M. Blau

summary/abstract:

Duchenne muscular dystrophy (DMD) is a common fatal heritable myopathy, with cardiorespiratory failure occurring by the third decade of life. There is no specific treatment for DMD cardiomyopathy, in large part due to a lack of understanding of the mechanisms underlying the cardiac failure. Mdx mice, which have the same dystrophin mutation as human patients, are of limited use, as they do not develop early dilated cardiomyopathy as seen in patients. Here we summarize the usefulness of the various commonly used DMD mouse models, highlight a model with shortened telomeres like humans, and identify directions that warrant further investigation.

organisation: Stanford University School of Medicine, USA; The Jackson Laboratory, Bar Harbor, USA

DOI: 10.1038/s41536-018-0045-4

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