{"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":1791614029675,"version":"4.3.4"},"reference-count":88,"publisher":"MDPI AG","issue":"7","license":[{"start":{"date-parts":[[2021,6,22]],"date-time":"2021-06-22T00:00:00Z","timestamp":1624320000000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"DOI":"10.13039\/501100001602","name":"Science Foundation Ireland","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>Extraocular muscles (EOMs) represent a specialized type of contractile tissue with unique cellular, physiological, and biochemical properties. In Duchenne muscular dystrophy, EOMs stay functionally unaffected in the course of disease progression. Therefore, it was of interest to determine their proteomic profile in dystrophinopathy. The proteomic survey of wild type mice and the dystrophic mdx-4cv model revealed a broad spectrum of sarcomere-associated proteoforms, including components of the thick filament, thin filament, M-band and Z-disk, as well as a variety of muscle-specific markers. Interestingly, the mass spectrometric analysis revealed unusual expression levels of contractile proteins, especially isoforms of myosin heavy chain. As compared to diaphragm muscle, both proteomics and immunoblotting established isoform MyHC14 as a new potential marker in wild type EOMs, in addition to the previously identified isoforms MyHC13 and MyHC15. Comparative proteomics was employed to establish alterations in the protein expression profile between normal EOMs and dystrophin-lacking EOMs. The analysis of mdx-4cv EOMs identified elevated levels of glycolytic enzymes and molecular chaperones, as well as decreases in mitochondrial enzymes. These findings suggest a process of adaptation in dystrophin-deficient EOMs via a bioenergetic shift to more glycolytic metabolism, as well as an efficient cellular stress response in EOMs in dystrophinopathy.<\/jats:p>","DOI":"10.3390\/life11070595","type":"journal-article","created":{"date-parts":[[2021,6,22]],"date-time":"2021-06-22T13:02:10Z","timestamp":1624366930000},"page":"595","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":12,"title":["Mass Spectrometric Profiling of Extraocular Muscle and Proteomic Adaptations in the mdx-4cv Model of Duchenne Muscular Dystrophy"],"prefix":"10.3390","volume":"11","author":[{"given":"Stephen","family":"Gargan","sequence":"first","affiliation":[{"name":"Department of Biology, National University of Ireland, Co. Kildare, W23F2H6 Maynooth, Ireland"},{"name":"Kathleen Lonsdale Institute for Human Health Research, Maynooth University, Co. Kildare, W23F2H6 Maynooth, Ireland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Paul","family":"Dowling","sequence":"additional","affiliation":[{"name":"Department of Biology, National University of Ireland, Co. Kildare, W23F2H6 Maynooth, Ireland"},{"name":"Kathleen Lonsdale Institute for Human Health Research, Maynooth University, Co. Kildare, W23F2H6 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, University of Bonn, D53113 Bonn, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-3349-6877","authenticated-orcid":false,"given":"Jens","family":"Reimann","sequence":"additional","affiliation":[{"name":"Department of Neurology, University Hospital, University of Bonn, D53113 Bonn, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Michael","family":"Henry","sequence":"additional","affiliation":[{"name":"National Institute for Cellular Biotechnology, Dublin City University, D09V209 Dublin, Ireland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Paula","family":"Meleady","sequence":"additional","affiliation":[{"name":"National Institute for Cellular Biotechnology, Dublin City University, D09V209 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 II, 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, National University of Ireland, Co. 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