{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,15]],"date-time":"2026-09-15T05:27:32Z","timestamp":1789450052224,"version":"build-2803163510"},"reference-count":36,"publisher":"Wiley","issue":"4","license":[{"start":{"date-parts":[[2013,12,17]],"date-time":"2013-12-17T00:00:00Z","timestamp":1387238400000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"funder":[{"DOI":"10.13039\/100000002","name":"National Institutes of Health","doi-asserted-by":"publisher","award":["RR\u2010030232"],"award-info":[{"award-number":["RR\u2010030232"]}],"id":[{"id":"10.13039\/100000002","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100000002","name":"National Institutes of Health","doi-asserted-by":"publisher","award":["NS\u2010079603"],"award-info":[{"award-number":["NS\u2010079603"]}],"id":[{"id":"10.13039\/100000002","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":[[2014,4]]},"abstract":"<jats:p>\n                    The purpose of this investigation was to determine the extent to which dystrophin insufficiency caused histomorphological changes in a novel pig model of Becker muscular dystrophy. In our procedures, we used a combination of biochemical approaches, including quantitative PCR and Western blots, along with a histological analysis using standard and immunohistological measures. We found that 8\u2010wk\u2010old male affected pigs had a 70% reduction in dystrophin protein abundance in the diaphragm, psoas major, and longissimus lumborum and a 5\u2010fold increase in serum creatine kinase activity compared with healthy male littermates. Dystrophin insufficiency in the diaphragm and the longissimus resulted in muscle histopathology with disorganized fibrosis that often colocalized with fatty infiltration but not the psoas. Affected animals also had an 80\u201385% reduction in \u03b1\u2010sarcoglycan localization in these muscles, indicating compromised assembly of the dystrophin glycoprotein complex. Controls used in this study were 4 healthy male littermates, as they are most closely related to the affected animals. We concluded that pigs with insufficient dystrophin protein expression have a phenotype consistent with human dystrophinopathy patients. Given that and their similarity in body size and physiology to humans, we further conclude that this pig line is an appropriate translational model for dystrophinopathies.\u2014Hollinger, K., Yang, C. X., Montz, R. E., Nonneman, D., Ross, J. W., Selsby, J. T. Dystrophin insufficiency causes selective muscle histopathology and loss of dystrophin\u2010glycoprotein complex assembly in pig skeletal muscle.\n                    <jats:italic>FASEB J.<\/jats:italic>\n                    28, 28\u20131600 (1609).\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.13-241141","type":"journal-article","created":{"date-parts":[[2013,12,17]],"date-time":"2013-12-17T23:18:50Z","timestamp":1387322330000},"page":"1600-1609","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":24,"title":["Dystrophin insufficiency causes selective muscle histopathology and loss of dystrophin\u2010glycoprotein complex assembly in pig skeletal muscle"],"prefix":"10.1096","volume":"28","author":[{"given":"Katrin","family":"Hollinger","sequence":"first","affiliation":[{"name":"Department of Animal Science Iowa State University Ames Iowa USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Cai X.","family":"Yang","sequence":"additional","affiliation":[{"name":"Department of Animal Science Iowa State University Ames Iowa USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Robyn E.","family":"Montz","sequence":"additional","affiliation":[{"name":"Department of Animal Science Iowa State University Ames Iowa USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Dan","family":"Nonneman","sequence":"additional","affiliation":[{"name":"U.S. Department of Agriculture, Agricultural Research Services U.S. Meat Animal Research Center Clay Center Nebraska USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jason W.","family":"Ross","sequence":"additional","affiliation":[{"name":"Department of Animal Science Iowa State University Ames Iowa USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Joshua T.","family":"Selsby","sequence":"additional","affiliation":[{"name":"Department of Animal Science Iowa State University Ames Iowa USA"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2013,12,17]]},"reference":[{"key":"e_1_2_7_2_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0070-2153(08)00609-1"},{"key":"e_1_2_7_3_1","doi-asserted-by":"publisher","DOI":"10.1096\/fj.06-7353com"},{"key":"e_1_2_7_4_1","doi-asserted-by":"publisher","DOI":"10.1242\/jcs.107.6.1477"},{"key":"e_1_2_7_5_1","doi-asserted-by":"publisher","DOI":"10.1093\/hmg\/9.9.1357"},{"key":"e_1_2_7_6_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0092-8674(00)80532-2"},{"key":"e_1_2_7_7_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.cell.2010.11.039"},{"key":"e_1_2_7_8_1","doi-asserted-by":"publisher","DOI":"10.1002\/mus.22318"},{"key":"e_1_2_7_9_1","first-page":"172","article-title":"What do animal models have to tell us regarding Duchenne muscular dystrophy?","volume":"24","author":"Wells D. 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