{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,6]],"date-time":"2026-07-06T18:40:32Z","timestamp":1783363232980,"version":"3.54.6"},"reference-count":35,"publisher":"MDPI AG","issue":"18","license":[{"start":{"date-parts":[[2022,9,16]],"date-time":"2022-09-16T00:00:00Z","timestamp":1663286400000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"DOI":"10.13039\/100000002","name":"the Emerging Talents Initiative of the Friedrich-Alexander University","doi-asserted-by":"publisher","award":["AR052646"],"award-info":[{"award-number":["AR052646"]}],"id":[{"id":"10.13039\/100000002","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100000002","name":"the Emerging Talents Initiative of the Friedrich-Alexander University","doi-asserted-by":"publisher","award":["57444393"],"award-info":[{"award-number":["57444393"]}],"id":[{"id":"10.13039\/100000002","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100000002","name":"the Emerging Talents Initiative of the Friedrich-Alexander University","doi-asserted-by":"publisher","award":["GSC 80"],"award-info":[{"award-number":["GSC 80"]}],"id":[{"id":"10.13039\/100000002","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100001655","name":"German Academic Exchange Service","doi-asserted-by":"publisher","award":["AR052646"],"award-info":[{"award-number":["AR052646"]}],"id":[{"id":"10.13039\/501100001655","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100001655","name":"German Academic Exchange Service","doi-asserted-by":"publisher","award":["57444393"],"award-info":[{"award-number":["57444393"]}],"id":[{"id":"10.13039\/501100001655","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100001655","name":"German Academic Exchange Service","doi-asserted-by":"publisher","award":["GSC 80"],"award-info":[{"award-number":["GSC 80"]}],"id":[{"id":"10.13039\/501100001655","id-type":"DOI","asserted-by":"publisher"}]},{"name":"the Erlangen Graduate School in Advanced Optical Technologies","award":["AR052646"],"award-info":[{"award-number":["AR052646"]}]},{"name":"the Erlangen Graduate School in Advanced Optical Technologies","award":["57444393"],"award-info":[{"award-number":["57444393"]}]},{"name":"the Erlangen Graduate School in Advanced Optical Technologies","award":["GSC 80"],"award-info":[{"award-number":["GSC 80"]}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["IJMS"],"abstract":"<jats:p>Duchenne muscular dystrophy (DMD) is a degenerative genetic myopathy characterized by complete absence of dystrophin. Although the mdx mouse lacks dystrophin, its phenotype is milder compared to DMD patients. The incorporation of a null mutation in the Cmah gene led to a more DMD-like phenotype (i.e., more fibrosis). Although fibrosis is thought to be the major determinant of \u2018structural weakness\u2019, intracellular remodeling of myofibrillar geometry was shown to be a major cellular determinant thereof. To dissect the respective contribution to muscle weakness, we assessed biomechanics and extra- and intracellular architecture of whole muscle and single fibers from extensor digitorum longus (EDL) and diaphragm. Despite increased collagen contents in both muscles, passive stiffness in mdx Cmah\u2212\/\u2212 diaphragm was similar to wt mice (EDL muscles were twice as stiff). Isometric twitch and tetanic stresses were 50% reduced in mdx Cmah\u2212\/\u2212 diaphragm (15% in EDL). Myofibrillar architecture was severely compromised in mdx Cmah\u2212\/\u2212 single fibers of both muscle types, but more pronounced in diaphragm. Our results show that the mdx Cmah\u2212\/\u2212 genotype reproduces DMD-like fibrosis but is not associated with changes in passive visco-elastic muscle stiffness. Furthermore, detriments in active isometric force are compatible with the pronounced myofibrillar disarray of the dystrophic background.<\/jats:p>","DOI":"10.3390\/ijms231810841","type":"journal-article","created":{"date-parts":[[2022,9,18]],"date-time":"2022-09-18T23:39:22Z","timestamp":1663544362000},"page":"10841","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":7,"title":["Myofibrillar Lattice Remodeling Is a Structural Cytoskeletal Predictor of Diaphragm Muscle Weakness in a Fibrotic mdx (mdx Cmah\u2212\/\u2212) Model"],"prefix":"10.3390","volume":"23","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-9227-8031","authenticated-orcid":false,"given":"Paul","family":"Ritter","sequence":"first","affiliation":[{"name":"Institute of Medical Biotechnology, Department of Chemical and Biological Engineering, Friedrich-Alexander-University Erlangen-N\u00fcrnberg, Paul-Gordan-Str. 3, 91052 Erlangen, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Stefanie","family":"N\u00fcbler","sequence":"additional","affiliation":[{"name":"Institute of Medical Biotechnology, Department of Chemical and Biological Engineering, Friedrich-Alexander-University Erlangen-N\u00fcrnberg, Paul-Gordan-Str. 3, 91052 Erlangen, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-5065-5430","authenticated-orcid":false,"given":"Andreas","family":"Buttgereit","sequence":"additional","affiliation":[{"name":"Institute of Medical Biotechnology, Department of Chemical and Biological Engineering, Friedrich-Alexander-University Erlangen-N\u00fcrnberg, Paul-Gordan-Str. 3, 91052 Erlangen, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-7610-4231","authenticated-orcid":false,"given":"Lucas R.","family":"Smith","sequence":"additional","affiliation":[{"name":"Department of Neurobiology, Physiology and Behavior, University of California, Davis, CA 95618, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Alexander","family":"M\u00fchlberg","sequence":"additional","affiliation":[{"name":"Institute of Medical Biotechnology, Department of Chemical and Biological Engineering, Friedrich-Alexander-University Erlangen-N\u00fcrnberg, Paul-Gordan-Str. 3, 91052 Erlangen, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Julian","family":"Bauer","sequence":"additional","affiliation":[{"name":"Institute of Medical Biotechnology, Department of Chemical and Biological Engineering, Friedrich-Alexander-University Erlangen-N\u00fcrnberg, Paul-Gordan-Str. 3, 91052 Erlangen, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-4805-0333","authenticated-orcid":false,"given":"Mena","family":"Michael","sequence":"additional","affiliation":[{"name":"Institute of Medical Biotechnology, Department of Chemical and Biological Engineering, Friedrich-Alexander-University Erlangen-N\u00fcrnberg, Paul-Gordan-Str. 3, 91052 Erlangen, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-7749-0015","authenticated-orcid":false,"given":"Lucas","family":"Krei\u00df","sequence":"additional","affiliation":[{"name":"Institute of Medical Biotechnology, Department of Chemical and Biological Engineering, Friedrich-Alexander-University Erlangen-N\u00fcrnberg, Paul-Gordan-Str. 3, 91052 Erlangen, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-5968-2593","authenticated-orcid":false,"given":"Michael","family":"Haug","sequence":"additional","affiliation":[{"name":"Institute of Medical Biotechnology, Department of Chemical and Biological Engineering, Friedrich-Alexander-University Erlangen-N\u00fcrnberg, Paul-Gordan-Str. 3, 91052 Erlangen, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Elisabeth","family":"Barton","sequence":"additional","affiliation":[{"name":"College of Health & Human Performance, University of Florida, Gainesville, FL 32611, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Oliver","family":"Friedrich","sequence":"additional","affiliation":[{"name":"Institute of Medical Biotechnology, Department of Chemical and Biological Engineering, Friedrich-Alexander-University Erlangen-N\u00fcrnberg, Paul-Gordan-Str. 3, 91052 Erlangen, Germany"},{"name":"School of Medical Sciences, University of New South Wales, Wallace Wurth Building, 18 High Str., Sydney, NSW 2052, Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2022,9,16]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"675","DOI":"10.1016\/0092-8674(95)90344-5","article-title":"Three muscular dystrophies: Review loss of 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