{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,16]],"date-time":"2026-09-16T19:20:43Z","timestamp":1789586443468,"version":"build-2803163510"},"reference-count":42,"publisher":"Wiley","issue":"5","license":[{"start":{"date-parts":[[2021,8,25]],"date-time":"2021-08-25T00:00:00Z","timestamp":1629849600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc\/4.0\/"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Muscle and Nerve"],"published-print":{"date-parts":[[2021,11]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Introduction\/Aims<\/jats:title><jats:p>Duchenne and Becker muscular dystrophies (DMD and BMD, respectively) are characterized by fat replacement of different skeletal muscles in a specific temporal order. Given the structural role of dystrophin in skeletal muscle mechanics, muscle architecture could be important in the progressive pathophysiology of muscle degeneration. Therefore, the aim of this study was to assess the role of muscle architecture in the progression of fat replacement in DMD and BMD.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>We assessed the association between literature\u2010based leg muscle architectural characteristics and muscle fat fraction from 22 DMD and 24 BMD patients. Dixon\u2010based magnetic resonance imaging estimates of fat fractions at baseline and 12 (only DMD) and 24\u2009months were related to fiber length and physiological cross\u2010sectional area (PCSA) using age\u2010controlled linear mixed modeling.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>DMD and BMD muscles with long fibers and BMD muscles with large PCSAs were associated with increased fat fraction. The effect of fiber length was stronger in muscles with larger PCSA.<\/jats:p><\/jats:sec><jats:sec><jats:title>Discussion<\/jats:title><jats:p>Muscle architecture may explain the pathophysiology of muscle degeneration in dystrophinopathies, in which proximal muscles with a larger mass (fiber length\u2009\u00d7\u2009PCSA) are more susceptible, confirming the clinical observation of a temporal proximal\u2010to\u2010distal progression. These results give more insight into the mechanical role in the pathophysiology of muscular dystrophies. Ultimately, this new information can be used to help support the selection of current and the development of future therapies.<\/jats:p><\/jats:sec>","DOI":"10.1002\/mus.27399","type":"journal-article","created":{"date-parts":[[2021,8,12]],"date-time":"2021-08-12T15:00:16Z","timestamp":1628780416000},"page":"576-584","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":20,"title":["Muscle architecture is associated with muscle fat replacement in <scp>Duchenne<\/scp> and <scp>Becker<\/scp> muscular dystrophies"],"prefix":"10.1002","volume":"64","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-8153-8862","authenticated-orcid":false,"given":"Thom T. J.","family":"Veeger","sequence":"first","affiliation":[{"name":"C.J. Gorter Center for High Field MRI, Department of Radiology Leiden University Medical Center  Leiden The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-5537-3179","authenticated-orcid":false,"given":"Erik W.","family":"van Zwet","sequence":"additional","affiliation":[{"name":"Department of Biostatistics Leiden University Medical Center  Leiden The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-8810-8571","authenticated-orcid":false,"given":"Diaa","family":"al Mohamad","sequence":"additional","affiliation":[{"name":"Department of Biostatistics Leiden University Medical Center  Leiden The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-5022-3745","authenticated-orcid":false,"given":"Karin J.","family":"Naarding","sequence":"additional","affiliation":[{"name":"Department of Neurology Leiden University Medical Center  Leiden The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-1541-130X","authenticated-orcid":false,"given":"Nienke M.","family":"van de Velde","sequence":"additional","affiliation":[{"name":"Department of Neurology Leiden University Medical Center  Leiden The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-2233-1383","authenticated-orcid":false,"given":"Melissa T.","family":"Hooijmans","sequence":"additional","affiliation":[{"name":"Department of Radiology &amp; Nuclear Medicine Amsterdam University Medical Centers, Location AMC  Amsterdam The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-4045-9732","authenticated-orcid":false,"given":"Andrew G.","family":"Webb","sequence":"additional","affiliation":[{"name":"C.J. 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