Disease course in mdx:utrophin+/− mice: comparison of three mouse models of Duchenne muscular dystrophy

@inproceedings{McDonald2015DiseaseCI,
  title={Disease course in mdx:utrophin+/− mice: comparison of three mouse models of Duchenne muscular dystrophy},
  author={Abby Ann McDonald and Sadie L. Hebert and Matthew D. Kunz and Steven J Ralles and Linda K McLoon},
  booktitle={Physiological reports},
  year={2015}
}
The mdx mouse model of Duchenne muscular dystrophy (DMD) is used to study disease mechanisms and potential treatments, but its pathology is less severe than DMD patients. Other mouse models were developed to more closely mimic the human disease based on knowledge that upregulation of utrophin has a protective effect in mdx muscle. An mdx:utrophin(-/-) (dko) mouse was created, which had a severe disease phenotype and a shortened life span. An mdx:utrophin(+/-) mouse was also created, which had… CONTINUE READING
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