{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,1]],"date-time":"2026-10-01T04:20:22Z","timestamp":1790828422840,"version":"4.1.0"},"reference-count":38,"publisher":"Wiley","issue":"7","license":[{"start":{"date-parts":[[2019,4,1]],"date-time":"2019-04-01T00:00:00Z","timestamp":1554076800000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"DOI":"10.13039\/501100007423","name":"Duchenne Parent Project","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100007423","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100013465","name":"French Muscular Dystrophy Association","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100013465","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100002426","name":"Fondazione Telethon","doi-asserted-by":"publisher","award":["20251"],"award-info":[{"award-number":["20251"]}],"id":[{"id":"10.13039\/501100002426","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100001826","name":"ZonMw","doi-asserted-by":"publisher","award":["113302001"],"award-info":[{"award-number":["113302001"]}],"id":[{"id":"10.13039\/501100001826","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100005039","name":"Leids Universitair Medisch Centrum","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100005039","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100013223","name":"PTC Therapeutics","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100013223","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["faseb.onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["The FASEB Journal"],"published-print":{"date-parts":[[2019,7]]},"abstract":"<jats:title>ABSTRACT<\/jats:title>\n                  <jats:p>\n                    The C57BL\/10ScSn\u2010\n                    <jats:italic>\n                      Dmd\n                      <jats:sup>mdx<\/jats:sup>\n                    <\/jats:italic>\n                    \/J (BL10\n                    <jats:italic>\u2010mdx<\/jats:italic>\n                    ) mouse has been the most commonly used model for Duchenne muscular dystrophy (DMD) for decades. Their muscle dysfunction and pathology is, however, less severe than in patients with DMD, which complicates preclinical studies. Recent discoveries indicate that disease severity is exacerbated when muscular dystrophy mouse models are generated on a DBA2\/J genetic background. Knowledge on the natural history of animal models is pivotal for high\u2010quality preclinical testing. However, for BL10\n                    <jats:italic>\u2010mdx<\/jats:italic>\n                    mice on a DBA2\/J background (D2\n                    <jats:italic>\u2010mdx<\/jats:italic>\n                    ), limited data are available. We addressed this gap in the natural history knowledge. First, we compared histopathological aspects in skeletal muscles of young D2\n                    <jats:italic>\u2010mdx<\/jats:italic>\n                    , BL10\n                    <jats:italic>\u2010mdx<\/jats:italic>\n                    , and wild\u2010type mice. Pathology was more pronounced in D2\n                    <jats:italic>\u2010mdx<\/jats:italic>\n                    mice and differed in severity between muscles within individuals. Secondly, we subjected D2\u2010\n                    <jats:italic>mdx<\/jats:italic>\n                    mice to a functional test regime for 34 weeks and identified that female D2\u2010\n                    <jats:italic>mdx<\/jats:italic>\n                    mice outperform severely impaired males, making females less useful for functional preclinical studies. Direct comparisons between 10\u2010 and 34\u2010wk\u2010old D2\n                    <jats:italic>\u2010mdx<\/jats:italic>\n                    mice revealed that disease pathology ameliorates with age. Heart pathology was progressive, with some features already evident at a young age. This natural history study of the D2\u2010\n                    <jats:italic>mdx<\/jats:italic>\n                    mouse will be instrumental for experimental design of future preclinical studies.\u2014Van Putten, M., Putker, K., Overzier, M., Adamzek, W. A., Pasteuning\u2010Vuhman, S., Plomp, J. J., Aartsma\u2010Rus, A. Natural disease history of the D2\u2010\n                    <jats:italic>mdx<\/jats:italic>\n                    mouse model for Duchenne muscular dystrophy. FASEB J. 33, 8110\u20138124 (2019).\n                    <jats:ext-link xmlns:xlink=\"http:\/\/www.w3.org\/1999\/xlink\" xlink:href=\"http:\/\/www.fasebj.org\">www.fasebj.org<\/jats:ext-link>\n                  <\/jats:p>","DOI":"10.1096\/fj.201802488r","type":"journal-article","created":{"date-parts":[[2019,4,1]],"date-time":"2019-04-01T19:02:04Z","timestamp":1554145324000},"page":"8110-8124","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":142,"title":["Natural disease history of the\n                    <i>D2\u2010mdx<\/i>\n                    mouse model for Duchenne muscular dystrophy"],"prefix":"10.1096","volume":"33","author":[{"given":"Maaike","family":"van Putten","sequence":"first","affiliation":[{"name":"Department of Human Genetics and Leiden University Medical Centre  Leiden The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kayleigh","family":"Putker","sequence":"additional","affiliation":[{"name":"Department of Human Genetics and Leiden University Medical Centre  Leiden The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Maurice","family":"Overzier","sequence":"additional","affiliation":[{"name":"Department of Human Genetics and Leiden University Medical Centre  Leiden The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"W. A.","family":"Adamzek","sequence":"additional","affiliation":[{"name":"Department of Human Genetics and Leiden University Medical Centre  Leiden The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Svetlana","family":"Pasteuning-Vuhman","sequence":"additional","affiliation":[{"name":"Department of Human Genetics and Leiden University Medical Centre  Leiden The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jaap J.","family":"Plomp","sequence":"additional","affiliation":[{"name":"Department of Neurology Leiden University Medical Centre  Leiden The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Annemieke","family":"Aartsma-Rus","sequence":"additional","affiliation":[{"name":"Department of Human Genetics and Leiden University Medical Centre  Leiden The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2019,4]]},"reference":[{"key":"e_1_2_8_2_1","doi-asserted-by":"publisher","DOI":"10.1002\/ana.23982"},{"key":"e_1_2_8_3_1","doi-asserted-by":"publisher","DOI":"10.1038\/ejhg.2012.301"},{"key":"e_1_2_8_4_1","doi-asserted-by":"publisher","DOI":"10.1016\/S1474-4422(16)30035-7"},{"key":"e_1_2_8_5_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0140-6736(02)07815-7"},{"key":"e_1_2_8_6_1","doi-asserted-by":"publisher","DOI":"10.1177\/0883073814558120"},{"key":"e_1_2_8_7_1","doi-asserted-by":"publisher","DOI":"10.1177\/0883073810371004"},{"key":"e_1_2_8_8_1","volume-title":"Duchenne MuscularDystrophy","author":"Emery A. 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