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Metabolites 2018, 8(4), 61; https://doi.org/10.3390/metabo8040061

Metabolomic Analyses Reveal Extensive Progenitor Cell Deficiencies in a Mouse Model of Duchenne Muscular Dystrophy

Department of Biochemistry and Molecular Biology, Mayo Clinic, Rochester, MN 55905, USA
These authors contributed equally to this work.
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Received: 18 August 2018 / Revised: 25 September 2018 / Accepted: 30 September 2018 / Published: 3 October 2018
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Abstract

Duchenne muscular dystrophy (DMD) is a musculoskeletal disorder that causes severe morbidity and reduced lifespan. Individuals with DMD have an X-linked mutation that impairs their ability to produce functional dystrophin protein in muscle. No cure exists for this disease and the few therapies that are available do not dramatically delay disease progression. Thus, there is a need to better understand the mechanisms underlying DMD which may ultimately lead to improved treatment options. The muscular dystrophy (MDX) mouse model is frequently used to explore DMD disease traits. Though some studies of metabolism in dystrophic mice exist, few have characterized metabolic profiles of supporting cells in the diseased environment. Using nontargeted metabolomics we characterized metabolic alterations in muscle satellite cells (SCs) and serum of MDX mice. Additionally, live-cell imaging revealed MDX-derived adipose progenitor cell (APC) defects. Finally, metabolomic studies revealed a striking elevation of acylcarnitines in MDX APCs, which we show can inhibit APC proliferation. Together, these studies highlight widespread metabolic alterations in multiple progenitor cell types and serum from MDX mice and implicate dystrophy-associated metabolite imbalances in APCs as a potential contributor to adipose tissue disequilibrium in DMD. View Full-Text
Keywords: metabolomics; Duchenne muscular dystrophy; stem cells; skeletal muscle; adipose tissue metabolomics; Duchenne muscular dystrophy; stem cells; skeletal muscle; adipose tissue
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Joseph, J.; Cho, D.S.; Doles, J.D. Metabolomic Analyses Reveal Extensive Progenitor Cell Deficiencies in a Mouse Model of Duchenne Muscular Dystrophy. Metabolites 2018, 8, 61.

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