Home > Publications Database > Extracellular Matrix Proteomics: The mdx-4cv Mouse Diaphragm as a Surrogate for Studying Myofibrosis in Dystrophinopathy. > print

001     259687
005     20231120155352.0
024 7 _ |a 10.3390/biom13071108
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024 7 _ |a pmc:PMC10377647
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037 _ _ |a DZNE-2023-00759
041 _ _ |a English
082 _ _ |a 570
100 1 _ |a Dowling, Paul
|b 0
245 _ _ |a Extracellular Matrix Proteomics: The mdx-4cv Mouse Diaphragm as a Surrogate for Studying Myofibrosis in Dystrophinopathy.
260 _ _ |a Basel
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520 _ _ |a The progressive degeneration of the skeletal musculature in Duchenne muscular dystrophy is accompanied by reactive myofibrosis, fat substitution, and chronic inflammation. Fibrotic changes and reduced tissue elasticity correlate with the loss in motor function in this X-chromosomal disorder. Thus, although dystrophinopathies are due to primary abnormalities in the DMD gene causing the almost-complete absence of the cytoskeletal Dp427-M isoform of dystrophin in voluntary muscles, the excessive accumulation of extracellular matrix proteins presents a key histopathological hallmark of muscular dystrophy. Animal model research has been instrumental in the characterization of dystrophic muscles and has contributed to a better understanding of the complex pathogenesis of dystrophinopathies, the discovery of new disease biomarkers, and the testing of novel therapeutic strategies. In this article, we review how mass-spectrometry-based proteomics can be used to study changes in key components of the endomysium, perimysium, and epimysium, such as collagens, proteoglycans, matricellular proteins, and adhesion receptors. The mdx-4cv mouse diaphragm displays severe myofibrosis, making it an ideal model system for large-scale surveys of systematic alterations in the matrisome of dystrophic fibers. Novel biomarkers of myofibrosis can now be tested for their appropriateness in the preclinical and clinical setting as diagnostic, pharmacodynamic, prognostic, and/or therapeutic monitoring indicators.
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650 _ 7 |a biglycan
|2 Other
650 _ 7 |a collagen
|2 Other
650 _ 7 |a dystrophin
|2 Other
650 _ 7 |a dystrophinopathy
|2 Other
650 _ 7 |a extracellular matrix
|2 Other
650 _ 7 |a fibronectin
|2 Other
650 _ 7 |a fibrosis
|2 Other
650 _ 7 |a matrisome
|2 Other
650 _ 7 |a mdx
|2 Other
650 _ 7 |a periostin
|2 Other
650 _ 7 |a Extracellular Matrix Proteins
|2 NLM Chemicals
650 _ 7 |a Biomarkers
|2 NLM Chemicals
650 _ 2 |a Animals
|2 MeSH
650 _ 2 |a Mice
|2 MeSH
650 _ 2 |a Mice, Inbred mdx
|2 MeSH
650 _ 2 |a Diaphragm: metabolism
|2 MeSH
650 _ 2 |a Diaphragm: pathology
|2 MeSH
650 _ 2 |a Proteomics
|2 MeSH
650 _ 2 |a Muscular Dystrophy, Duchenne: pathology
|2 MeSH
650 _ 2 |a Muscle, Skeletal: metabolism
|2 MeSH
650 _ 2 |a Extracellular Matrix: metabolism
|2 MeSH
650 _ 2 |a Extracellular Matrix Proteins: genetics
|2 MeSH
650 _ 2 |a Extracellular Matrix Proteins: metabolism
|2 MeSH
650 _ 2 |a Biomarkers: metabolism
|2 MeSH
700 1 _ |a Gargan, Stephen
|b 1
700 1 _ |a Zweyer, Margit
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700 1 _ |a Swandulla, Dieter
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700 1 _ |a Ohlendieck, Kay
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770 _ _ |a Muscular Dystrophy: From Molecular Basis to Therapies
773 _ _ |a 10.3390/biom13071108
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