% IMPORTANT: The following is UTF-8 encoded.  This means that in the presence
% of non-ASCII characters, it will not work with BibTeX 0.99 or older.
% Instead, you should use an up-to-date BibTeX implementation like “bibtex8” or
% “biber”.

@ARTICLE{Dowling:259687,
      author       = {Dowling, Paul and Gargan, Stephen and Zweyer, Margit and
                      Swandulla, Dieter and Ohlendieck, Kay},
      title        = {{E}xtracellular {M}atrix {P}roteomics: {T}he mdx-4cv
                      {M}ouse {D}iaphragm as a {S}urrogate for {S}tudying
                      {M}yofibrosis in {D}ystrophinopathy.},
      journal      = {Biomolecules},
      volume       = {13},
      number       = {7},
      issn         = {2218-273X},
      address      = {Basel},
      publisher    = {MDPI},
      reportid     = {DZNE-2023-00759},
      pages        = {1108},
      year         = {2023},
      abstract     = {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.},
      subtyp        = {Review Article},
      keywords     = {Animals / Mice / Mice, Inbred mdx / Diaphragm: metabolism /
                      Diaphragm: pathology / Proteomics / Muscular Dystrophy,
                      Duchenne: pathology / Muscle, Skeletal: metabolism /
                      Extracellular Matrix: metabolism / Extracellular Matrix
                      Proteins: genetics / Extracellular Matrix Proteins:
                      metabolism / Biomarkers: metabolism / biglycan (Other) /
                      collagen (Other) / dystrophin (Other) / dystrophinopathy
                      (Other) / extracellular matrix (Other) / fibronectin (Other)
                      / fibrosis (Other) / matrisome (Other) / mdx (Other) /
                      periostin (Other) / Extracellular Matrix Proteins (NLM
                      Chemicals) / Biomarkers (NLM Chemicals)},
      cin          = {AG Sabir},
      ddc          = {570},
      cid          = {I:(DE-2719)5000032},
      pnm          = {352 - Disease Mechanisms (POF4-352)},
      pid          = {G:(DE-HGF)POF4-352},
      typ          = {PUB:(DE-HGF)16},
      pubmed       = {pmid:37509144},
      pmc          = {pmc:PMC10377647},
      doi          = {10.3390/biom13071108},
      url          = {https://pub.dzne.de/record/259687},
}