{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2023,9,25]],"date-time":"2023-09-25T05:45:54Z","timestamp":1695620754675},"reference-count":24,"publisher":"Wiley","issue":"3","license":[{"start":{"date-parts":[[2015,12,29]],"date-time":"2015-12-29T00:00:00Z","timestamp":1451347200000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"funder":[{"name":"NIH"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Muscle and Nerve"],"published-print":{"date-parts":[[2016,3]]},"abstract":"<jats:title>ABSTRACT<\/jats:title><jats:p><jats:italic>Introduction<\/jats:italic>: Previous experiments have indicated that <jats:italic>in vivo<\/jats:italic> administration of ursodeoxycholic acid (UDCA) inhibits nuclear NF\u2010\u03baB activation and has beneficial effects on the structure and function of dystrophic (<jats:italic>mdx<\/jats:italic>) muscle. We examined the effect of UDCA on tension development in dystrophic muscle. <jats:italic>Methods<\/jats:italic>: Isometric tension development was examined in costal diaphragms that were freshly isolated from vehicle and UDCA treated <jats:italic>mdx<\/jats:italic> mice. Percent recovery scores were obtained by directly comparing these measurements to those obtained from age\u2010matched nondystrophic mice. <jats:italic>Results<\/jats:italic>: Vehicle treated <jats:italic>mdx<\/jats:italic> mice exhibited significantly reduced optimal muscle lengths (l<jats:sub>o<\/jats:sub>) and specific twitch and tetanic tensions compared with age\u2010matched nondystrophic mice. UDCA treated preparations exhibited significantly improved tension development with a 33% recovery score. <jats:italic>Conclusions<\/jats:italic>: Because UDCA is used in treating certain clinical disorders, these results provide a rationale for human clinical trials using this and related drugs for treatment of Duchenne and related muscular dystrophies. <jats:italic>Muscle Nerve<\/jats:italic> 53: 431\u2013437, 2016<\/jats:p>","DOI":"10.1002\/mus.24755","type":"journal-article","created":{"date-parts":[[2015,7,4]],"date-time":"2015-07-04T05:58:26Z","timestamp":1435989506000},"page":"431-437","source":"Crossref","is-referenced-by-count":3,"title":["<i>In vivo<\/i> treatment with the NF\u2010\u03baB inhibitor ursodeoxycholic acid (UDCA) improves tension development in the isolated <i>mdx<\/i> costal diaphragm"],"prefix":"10.1002","volume":"53","author":[{"given":"C. George","family":"Carlson","sequence":"first","affiliation":[{"name":"Department of Physiology Midwestern University Glendale Glendale Arizona 85308"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ross","family":"Potter","sequence":"additional","affiliation":[{"name":"Department of Physiology Midwestern University Glendale Glendale Arizona 85308"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Vivien","family":"Yu","sequence":"additional","affiliation":[{"name":"Department of Physiology Midwestern University Glendale Glendale Arizona 85308"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Kevin","family":"Luo","sequence":"additional","affiliation":[{"name":"Department of Physiology Midwestern University Glendale Glendale Arizona 85308"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jesse","family":"Lavin","sequence":"additional","affiliation":[{"name":"Department of Physiology Midwestern University Glendale Glendale Arizona 85308"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Cory","family":"Nielsen","sequence":"additional","affiliation":[{"name":"Department of Physiology Midwestern University Glendale Glendale Arizona 85308"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2015,12,29]]},"reference":[{"key":"e_1_2_7_2_1","doi-asserted-by":"publisher","DOI":"10.1212\/01.WNL.0000049913.27181.51"},{"key":"e_1_2_7_3_1","doi-asserted-by":"publisher","DOI":"10.1096\/fj.02-0542com"},{"key":"e_1_2_7_4_1","doi-asserted-by":"publisher","DOI":"10.1172\/JCI30556"},{"key":"e_1_2_7_5_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jns.2009.06.030"},{"key":"e_1_2_7_6_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.nbd.2005.05.003"},{"key":"e_1_2_7_7_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.expneurol.2005.11.021"},{"key":"e_1_2_7_8_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.nmd.2008.10.006"},{"key":"e_1_2_7_9_1","doi-asserted-by":"publisher","DOI":"10.1002\/ar.21294"},{"key":"e_1_2_7_10_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0168-8278(01)00092-7"},{"key":"e_1_2_7_11_1","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.M107098200"},{"key":"e_1_2_7_12_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jns.2011.01.015"},{"key":"e_1_2_7_13_1","doi-asserted-by":"publisher","DOI":"10.1093\/emboj\/cdg332"},{"key":"e_1_2_7_14_1","doi-asserted-by":"publisher","DOI":"10.1002\/mus.21944"},{"key":"e_1_2_7_15_1","doi-asserted-by":"publisher","DOI":"10.1113\/jphysiol.2007.148338"},{"key":"e_1_2_7_16_1","doi-asserted-by":"publisher","DOI":"10.1002\/mus.20579"},{"key":"e_1_2_7_17_1","unstructured":"GillisJP.The recovery score to evaluate therapy efficiency in NMD: a common quantitative and comparative scoring system.http:\/\/www.treat\u2010nmd.eu\/research\/preclinical\/dmd\u2010sops\/2008; 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