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Sci Rep. 2018 Sep 11;8(1):13624. doi: 10.1038/s41598-018-31753-3.

Integrative effects of dystrophin loss on metabolic function of the mdx mouse.

Author information

1
Department of Integrative Biology and Physiology, Medical School, University of Minnesota, Minneapolis, MN, USA.
2
Department of Medicine, Cardiovascular Division, Medical School, University of Minnesota, Minneapolis, MN, USA.
3
Department of Biochemistry, Molecular Biology, and Biophysics, Medical School, University of Minnesota, Minneapolis, MN, USA.
4
Department of Integrative Biology and Physiology, Medical School, University of Minnesota, Minneapolis, MN, USA. town0045@umn.edu.

Abstract

Duchenne muscular dystrophy (DMD) is a disease marked by the development of skeletal muscle weakness and wasting. DMD results from mutations in the gene for the cytoskeletal protein dystrophin. The loss of dystrophin expression is not limited to muscle weakness but has multiple systemic consequences. Managing the nutritional requirements is an important aspect of the clinical care of DMD patients and is complicated by the poor understanding of the role of dystrophin, and dystrophic processes, in regulating metabolism. Here, we show that mdx mice, a genetic model of DMD, have significantly reduced fat mass relative to wild type C57BL/10. The alteration in body composition is independent of the presence of skeletal muscle disease, as it is still present in mice with transgenic expression of a fully-functional dystrophin in skeletal muscle. Furthermore, mdx mice do not increase their fat mass or body weight when housed under thermoneutral conditions, in marked contrast to C57BL/10 mice. We also demonstrated that mdx mice have significantly reduced fat metabolism and altered glucose uptake. These significant metabolic changes in dystrophic mice implicate dystrophin as an important regulator of metabolism. Understanding the metabolic functions of dystrophin is important for managing the nutritional needs of DMD patients.

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