{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,10]],"date-time":"2026-10-10T06:33:49Z","timestamp":1791614029651,"version":"4.3.4"},"reference-count":138,"publisher":"MDPI AG","issue":"11","license":[{"start":{"date-parts":[[2022,10,22]],"date-time":"2022-10-22T00:00:00Z","timestamp":1666396800000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"name":"Kathleen Lonsdale Institute for Human Health Research at Maynooth University","award":["SFI 16\/RI\/3701"],"award-info":[{"award-number":["SFI 16\/RI\/3701"]}]},{"DOI":"10.13039\/501100001602","name":"Science Foundation Ireland Infrastructure Award to Dublin City University","doi-asserted-by":"publisher","award":["SFI 16\/RI\/3701"],"award-info":[{"award-number":["SFI 16\/RI\/3701"]}],"id":[{"id":"10.13039\/501100001602","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Life"],"abstract":"<jats:p>Deficiency in the membrane cytoskeletal protein dystrophin is the underlying cause of the progressive muscle wasting disease named Duchenne muscular dystrophy. In order to detect novel disease marker candidates and confirm the complexity of the pathobiochemical signature of dystrophinopathy, mass spectrometric screening approaches represent ideal tools for comprehensive biomarker discovery studies. In this report, we describe the comparative proteomic analysis of young versus aged diaphragm muscles from wild type versus the dystrophic mdx-4cv mouse model of X-linked muscular dystrophy. The survey confirmed the drastic reduction of the dystrophin-glycoprotein complex in the mdx-4cv diaphragm muscle and concomitant age-dependent changes in key markers of muscular dystrophy, including proteins involved in cytoskeletal organization, metabolite transportation, the cellular stress response and excitation-contraction coupling. Importantly, proteomic markers of the regulation of membrane repair, tissue regeneration and reactive myofibrosis were detected by mass spectrometry and changes in key proteins were confirmed by immunoblotting. Potential disease marker candidates include various isoforms of annexin, the matricellular protein periostin and a large number of collagens. Alterations in these proteoforms can be useful to evaluate adaptive, compensatory and pathobiochemical changes in the intracellular cytoskeleton, myofiber membrane integrity and the extracellular matrix in dystrophin-deficient skeletal muscle tissues.<\/jats:p>","DOI":"10.3390\/life12111679","type":"journal-article","created":{"date-parts":[[2022,10,24]],"date-time":"2022-10-24T04:40:36Z","timestamp":1666586436000},"page":"1679","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":16,"title":["Proteomic Identification of Markers of Membrane Repair, Regeneration and Fibrosis in the Aged and Dystrophic Diaphragm"],"prefix":"10.3390","volume":"12","author":[{"given":"Stephen","family":"Gargan","sequence":"first","affiliation":[{"name":"Department of Biology, Maynooth University, National University of Ireland, W23 F2H6 Maynooth, Ireland"},{"name":"Kathleen Lonsdale Institute for Human Health Research, Maynooth University, W23 F2H6 Maynooth, Ireland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Paul","family":"Dowling","sequence":"additional","affiliation":[{"name":"Department of Biology, Maynooth University, National University of Ireland, W23 F2H6 Maynooth, Ireland"},{"name":"Kathleen Lonsdale Institute for Human Health Research, Maynooth University, W23 F2H6 Maynooth, Ireland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Margit","family":"Zweyer","sequence":"additional","affiliation":[{"name":"Department of Neonatology and Paediatric Intensive Care, Children\u2019s Hospital, German Center for Neurodegenerative Diseases, University of Bonn, D53127 Bonn, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Michael","family":"Henry","sequence":"additional","affiliation":[{"name":"National Institute for Cellular Biotechnology, Dublin City University, D09 E432 Dublin, Ireland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Paula","family":"Meleady","sequence":"additional","affiliation":[{"name":"National Institute for Cellular Biotechnology, Dublin City University, D09 E432 Dublin, Ireland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-0923-7090","authenticated-orcid":false,"given":"Dieter","family":"Swandulla","sequence":"additional","affiliation":[{"name":"Institute of Physiology, University of Bonn, D53115 Bonn, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-6266-4510","authenticated-orcid":false,"given":"Kay","family":"Ohlendieck","sequence":"additional","affiliation":[{"name":"Department of Biology, Maynooth University, National University of Ireland, W23 F2H6 Maynooth, Ireland"},{"name":"Kathleen Lonsdale Institute for Human Health Research, Maynooth University, W23 F2H6 Maynooth, Ireland"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2022,10,22]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"980","DOI":"10.1016\/j.nmd.2019.10.010","article-title":"The 2020 version of the gene table of neuromuscular disorders","volume":"29","author":"Benarroch","year":"2019","journal-title":"Neuromuscul. 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