1. Humanizing the mdx mouse model of DMD: the long and the short of it
- Author
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John W. Day, Nora Yucel, Nadia Rosenthal, Helen M. Blau, and Alex C.Y. Chang
- Subjects
0301 basic medicine ,musculoskeletal diseases ,mdx mouse ,Technology ,congenital, hereditary, and neonatal diseases and abnormalities ,TELOMERE DYSFUNCTION ,Duchenne muscular dystrophy ,Biomedical Engineering ,Cardiomyopathy ,Medicine (miscellaneous) ,lcsh:Medicine ,ALPHA-7-BETA-1 INTEGRIN ,Review Article ,Bioinformatics ,DEFICIENT MICE ,DUCHENNE MUSCULAR-DYSTROPHY ,03 medical and health sciences ,ADULT SKELETAL-MUSCLE ,0302 clinical medicine ,Engineering ,Cell & Tissue Engineering ,SATELLITE CELLS ,medicine ,In patient ,Myopathy ,Author Correction ,Engineering, Biomedical ,Science & Technology ,biology ,business.industry ,lcsh:R ,GAMMA-SARCOGLYCAN ,BETA-DYSTROGLYCAN ,Dilated cardiomyopathy ,Cell Biology ,medicine.disease ,ALPHA-DYSTROBREVIN ,030104 developmental biology ,Cardiorespiratory failure ,biology.protein ,medicine.symptom ,business ,Dystrophin ,GLYCOPROTEIN COMPLEX ,Life Sciences & Biomedicine ,030217 neurology & neurosurgery ,Developmental Biology - 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.
- Published
- 2018