{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,17]],"date-time":"2026-09-17T22:12:51Z","timestamp":1789683171199,"version":"4.0.1"},"reference-count":14,"publisher":"Wiley","issue":"2","license":[{"start":{"date-parts":[[2011,1,19]],"date-time":"2011-01-19T00:00:00Z","timestamp":1295395200000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Muscle and Nerve"],"published-print":{"date-parts":[[2011,2]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>Muscle fibrosis is a prominent pathological feature that directly causes muscle dysfunction in Duchenne muscular dystrophy (DMD). The DMD mouse models, <jats:italic>mdx<\/jats:italic> mice and <jats:italic>mdx<\/jats:italic> mice with haploinsufficiency of the utrophin gene (<jats:italic>mdx\/utrn<\/jats:italic><jats:sup><jats:italic>+\/\u2212<\/jats:italic><\/jats:sup>), display progressive diaphragm fibrosis. We performed unrestrained whole\u2010body plethysmography (WBP) in <jats:italic>mdx<\/jats:italic> and <jats:italic>mdx\/utrn<\/jats:italic><jats:sup><jats:italic>+\/\u2212<\/jats:italic><\/jats:sup> mice, and compared them with wild\u2010type controls. Respiratory function gauged by respiratory frequency, tidal volume, minute volume, peak inspiratory flow, and peak expiratory flow was significantly impaired in the <jats:italic>mdx<\/jats:italic> mice. Consistent with more severe diaphragm fibrosis in the <jats:italic>mdx\/utrn<\/jats:italic><jats:sup><jats:italic>+\/\u2212<\/jats:italic><\/jats:sup> mice, respiratory impairment was worse than in <jats:italic>mdx<\/jats:italic> mice at 6 months. WBP is useful for monitoring in vivo respiratory function of <jats:italic>mdx<\/jats:italic> and <jats:italic>mdx\/utrn<\/jats:italic><jats:sup><jats:italic>+\/\u2212<\/jats:italic><\/jats:sup> mice, and it may serve as an outcome measurement for therapies that target diaphragm fibrosis. The <jats:italic>mdx\/utrn<\/jats:italic><jats:sup><jats:italic>+\/\u2212<\/jats:italic><\/jats:sup> mouse model may be better than the <jats:italic>mdx<\/jats:italic> model for testing antifibrotic therapies, especially at the severe stage. Muscle Nerve 43: 263\u2013267, 2011<\/jats:p>","DOI":"10.1002\/mus.21848","type":"journal-article","created":{"date-parts":[[2011,1,19]],"date-time":"2011-01-19T19:40:02Z","timestamp":1295466002000},"page":"263-267","source":"Crossref","is-referenced-by-count":71,"title":["Impaired respiratory function in <i>mdx<\/i> and <i>mdx\/utrn<\/i><sup><i>+\/\u2212<\/i><\/sup> mice"],"prefix":"10.1002","volume":"43","author":[{"given":"Ping","family":"Huang","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Georgiana","family":"Cheng","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Haiyan","family":"Lu","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mark","family":"Aronica","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Richard M.","family":"Ransohoff","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lan","family":"Zhou","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2011,1,19]]},"reference":[{"key":"e_1_2_6_2_2","doi-asserted-by":"publisher","DOI":"10.1016\/0960-8966(91)90039-U"},{"key":"e_1_2_6_3_2","volume-title":"Duchenne muscular dystrophy","author":"Emery A.","year":"1993"},{"key":"e_1_2_6_4_2","doi-asserted-by":"publisher","DOI":"10.1002\/mus.880151008"},{"key":"e_1_2_6_5_2","doi-asserted-by":"publisher","DOI":"10.1038\/352536a0"},{"key":"e_1_2_6_6_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.nmd.2005.09.009"},{"key":"e_1_2_6_7_2","doi-asserted-by":"publisher","DOI":"10.1002\/mus.10431"},{"key":"e_1_2_6_8_2","doi-asserted-by":"publisher","DOI":"10.1016\/S0960-8966(00)00170-X"},{"key":"e_1_2_6_9_2","doi-asserted-by":"publisher","DOI":"10.1016\/S0092-8674(00)80532-2"},{"key":"e_1_2_6_10_2","doi-asserted-by":"publisher","DOI":"10.1016\/S0092-8674(00)80533-4"},{"key":"e_1_2_6_11_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.jns.2007.08.029"},{"key":"e_1_2_6_12_2","doi-asserted-by":"publisher","DOI":"10.1038\/nm1536"},{"key":"e_1_2_6_13_2","doi-asserted-by":"publisher","DOI":"10.1096\/fj.09-129833"},{"key":"e_1_2_6_14_2","doi-asserted-by":"publisher","DOI":"10.1007\/s10974-007-9101-1"},{"key":"e_1_2_6_15_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.nmd.2008.02.002"}],"container-title":["Muscle &amp; Nerve"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1002%2Fmus.21848","content-type":"unspecified","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1002%2Fmus.21848","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/mus.21848","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,10,9]],"date-time":"2023-10-09T12:09:55Z","timestamp":1696853395000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1002\/mus.21848"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2011,1,19]]},"references-count":14,"journal-issue":{"issue":"2","published-print":{"date-parts":[[2011,2]]}},"alternative-id":["10.1002\/mus.21848"],"URL":"https:\/\/doi.org\/10.1002\/mus.21848","archive":["Portico"],"relation":{},"ISSN":["0148-639X","1097-4598"],"issn-type":[{"value":"0148-639X","type":"print"},{"value":"1097-4598","type":"electronic"}],"subject":[],"published":{"date-parts":[[2011,1,19]]}}}