Integrative effects of dystrophin loss on metabolic function of the mdx mouse
| Autor: | James M. Ervasti, Forum Kamdar, Maria Razzoli, Jana Strakova, DeWayne Townsend, Debra Kulhanek, Daniel J. Garry, Alessandro Bartolomucci |
|---|---|
| Jazyk: | angličtina |
| Rok vydání: | 2018 |
| Předmět: |
0301 basic medicine
musculoskeletal diseases mdx mouse medicine.medical_specialty congenital hereditary and neonatal diseases and abnormalities Duchenne muscular dystrophy lcsh:Medicine Mice Transgenic Article Dystrophin Mice 03 medical and health sciences 0302 clinical medicine Internal medicine Genetic model medicine Animals Humans Muscular dystrophy Muscle Skeletal lcsh:Science Multidisciplinary biology business.industry lcsh:R Muscle weakness Skeletal muscle Lipid metabolism Muscular Dystrophy Animal medicine.disease musculoskeletal system 3. Good health Muscular Dystrophy Duchenne Disease Models Animal Glucose 030104 developmental biology Endocrinology medicine.anatomical_structure Gene Expression Regulation Mutation Body Composition Mice Inbred mdx biology.protein lcsh:Q medicine.symptom business 030217 neurology & neurosurgery Muscle Contraction |
| Zdroj: | Scientific Reports, Vol 8, Iss 1, Pp 1-10 (2018) Scientific Reports |
| ISSN: | 2045-2322 |
| DOI: | 10.1038/s41598-018-31753-3 |
| Popis: | 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. |
| Databáze: | OpenAIRE |
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