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000259687 1001_ $$aDowling, Paul$$b0
000259687 245__ $$aExtracellular Matrix Proteomics: The mdx-4cv Mouse Diaphragm as a Surrogate for Studying Myofibrosis in Dystrophinopathy.
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000259687 520__ $$aThe 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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000259687 650_7 $$2Other$$abiglycan
000259687 650_7 $$2Other$$acollagen
000259687 650_7 $$2Other$$adystrophin
000259687 650_7 $$2Other$$adystrophinopathy
000259687 650_7 $$2Other$$aextracellular matrix
000259687 650_7 $$2Other$$afibronectin
000259687 650_7 $$2Other$$afibrosis
000259687 650_7 $$2Other$$amatrisome
000259687 650_7 $$2Other$$amdx
000259687 650_7 $$2Other$$aperiostin
000259687 650_7 $$2NLM Chemicals$$aExtracellular Matrix Proteins
000259687 650_7 $$2NLM Chemicals$$aBiomarkers
000259687 650_2 $$2MeSH$$aAnimals
000259687 650_2 $$2MeSH$$aMice
000259687 650_2 $$2MeSH$$aMice, Inbred mdx
000259687 650_2 $$2MeSH$$aDiaphragm: metabolism
000259687 650_2 $$2MeSH$$aDiaphragm: pathology
000259687 650_2 $$2MeSH$$aProteomics
000259687 650_2 $$2MeSH$$aMuscular Dystrophy, Duchenne: pathology
000259687 650_2 $$2MeSH$$aMuscle, Skeletal: metabolism
000259687 650_2 $$2MeSH$$aExtracellular Matrix: metabolism
000259687 650_2 $$2MeSH$$aExtracellular Matrix Proteins: genetics
000259687 650_2 $$2MeSH$$aExtracellular Matrix Proteins: metabolism
000259687 650_2 $$2MeSH$$aBiomarkers: metabolism
000259687 7001_ $$aGargan, Stephen$$b1
000259687 7001_ $$0P:(DE-2719)9000835$$aZweyer, Margit$$b2$$udzne
000259687 7001_ $$00000-0003-0923-7090$$aSwandulla, Dieter$$b3
000259687 7001_ $$00000-0002-6266-4510$$aOhlendieck, Kay$$b4
000259687 770__ $$aMuscular Dystrophy: From Molecular Basis to Therapies
000259687 773__ $$0PERI:(DE-600)2701262-1$$a10.3390/biom13071108$$gVol. 13, no. 7, p. 1108 -$$n7$$p1108$$tBiomolecules$$v13$$x2218-273X$$y2023
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