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

Npj Regenerative Medicine
Nora YucelHelen M Blau

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.

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Citations

Jul 11, 2019·Journal of Muscle Research and Cell Motility·Dominic J Wells
Oct 18, 2019·Cell Stress & Chaperones·Daniela Sayuri MizobutiElaine Minatel
Sep 3, 2020·Acta Physiologica·Kiyoshi YoshiokaYusuke Ono
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Methods Mentioned

BETA
biopsies
electron microscopy
gene trap
single knockout
gene knockout
gene knockouts

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