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Assessing Functional Performance in the Mdx Mouse Model
Published on: March 27, 2014
Impaired respiratory function in mdx and mdx/utrn(+/-) mice
Ping Huang1, Georgiana Cheng, Haiyan Lu
1Department of Neurology, Cleveland Clinic, Cleveland, OH 44195, USA.
Muscle & Nerve
|January 22, 2011
Summary
Duchenne muscular dystrophy (DMD) causes diaphragm fibrosis and impairs respiratory function. Unrestrained whole-body plethysmography effectively monitors this dysfunction in mdx mouse models, aiding antifibrotic therapy development.
Area of Science:
- Biomedical research
- Respiratory physiology
- Musculoskeletal disorders
Background:
- Muscle fibrosis is a key pathology in Duchenne muscular dystrophy (DMD), leading to muscle dysfunction.
- DMD mouse models, including mdx and mdx/utrn(+/-) mice, exhibit progressive diaphragm fibrosis.
Purpose of the Study:
- To assess respiratory function in mdx and mdx/utrn(+/-) mice using unrestrained whole-body plethysmography (WBP).
- To evaluate the utility of WBP as an outcome measure for antifibrotic therapies targeting diaphragm fibrosis in DMD models.
Main Methods:
- Unrestrained whole-body plethysmography (WBP) was employed to measure respiratory parameters.
- Respiratory frequency, tidal volume, minute volume, peak inspiratory flow, and peak expiratory flow were analyzed.
- mdx and mdx/utrn(+/-) mice were compared with wild-type controls.
Main Results:
- Significant impairment in respiratory function was observed in mdx mice compared to controls.
- mdx/utrn(+/-) mice showed more severe respiratory impairment than mdx mice, correlating with greater diaphragm fibrosis.
- WBP successfully monitored in vivo respiratory function in these DMD models.
Conclusions:
- WBP is a valuable tool for monitoring respiratory dysfunction in mdx and mdx/utrn(+/-) mice.
- The mdx/utrn(+/-) mouse model may be superior to the mdx model for evaluating antifibrotic therapies, particularly in advanced disease stages.
