PPARδ modulation rescues mitochondrial fatty acid oxidation defects in the mdx model of muscular dystrophy
| Autor: | Ross Fredenburg, Matthew M. Goddeeris, Jennifer K Truong, Effie Tozzo, Dominique Stickens, Robert W. Shine, Eric L. Bell, Peter Dwyer, Lyndsay Olson |
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| Rok vydání: | 2019 |
| Předmět: |
musculoskeletal diseases
0301 basic medicine congenital hereditary and neonatal diseases and abnormalities Bioenergetics Duchenne muscular dystrophy Mitochondrion Myoblasts 03 medical and health sciences medicine Animals Myocyte PPAR delta Muscular dystrophy Molecular Biology biology Chemistry Fatty Acids Wild type Skeletal muscle Cell Biology musculoskeletal system medicine.disease Mitochondria Cell biology Mice Inbred C57BL Muscular Dystrophy Duchenne Disease Models Animal 030104 developmental biology medicine.anatomical_structure Mice Inbred mdx biology.protein Molecular Medicine Energy Metabolism Dystrophin Oxidation-Reduction |
| Zdroj: | Mitochondrion. 46:51-58 |
| ISSN: | 1567-7249 |
| Popis: | Duchenne muscular dystrophy (DMD) is a recessive, fatal X-linked disease that is characterized by progressive skeletal muscle wasting due to the absence of dystrophin, which is an a essential protein that bridges the inner cytoskeleton and extra-cellular matrix. This study set out to characterize the mitochondria in primary muscle satellite cell derived myoblasts from mdx mice and wild type control mice. Compared to wild type derived cells the mdx derived cells have reduced mitochondrial bioenergetics and have fewer mitochondria. Here, we demonstrate that a novel PPARδ modulator improves mitochondrial function in the mdx mice, which supports that modulating PPARδ may be therapeutically beneficial in DMD patients. |
| Databáze: | OpenAIRE |
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