{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,18]],"date-time":"2026-10-18T05:14:28Z","timestamp":1792300468861,"version":"4.4.0"},"reference-count":69,"publisher":"The Company of Biologists","issue":"12","license":[{"start":{"date-parts":[[2021,12,16]],"date-time":"2021-12-16T00:00:00Z","timestamp":1639612800000},"content-version":"vor","delay-in-days":15,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0"}],"funder":[{"DOI":"10.13039\/501100003042","name":"Else Kr\u00f6ner-Fresenius-Stiftung","doi-asserted-by":"crossref","award":["2018_T20"],"award-info":[{"award-number":["2018_T20"]}],"id":[{"id":"10.13039\/501100003042","id-type":"DOI","asserted-by":"crossref"}]},{"DOI":"10.13039\/100010665","name":"Marie Sk\u0142odowska-Curie Actions","doi-asserted-by":"crossref","award":["812660"],"award-info":[{"award-number":["812660"]}],"id":[{"id":"10.13039\/100010665","id-type":"DOI","asserted-by":"crossref"}]},{"DOI":"10.13039\/100010665","name":"Marie Sk\u0142odowska-Curie Actions","doi-asserted-by":"crossref","award":["760986"],"award-info":[{"award-number":["760986"]}],"id":[{"id":"10.13039\/100010665","id-type":"DOI","asserted-by":"crossref"}]},{"DOI":"10.13039\/501100005722","name":"Ludwig-Maximilians-Universit\u00e4t M\u00fcnchen","doi-asserted-by":"crossref","id":[{"id":"10.13039\/501100005722","id-type":"DOI","asserted-by":"crossref"}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2021,12,1]]},"abstract":"<jats:title>ABSTRACT<\/jats:title>\n                  <jats:p>Large-animal models for Duchenne muscular dystrophy (DMD) are crucial for the evaluation of diagnostic procedures and treatment strategies. Pigs cloned from male cells lacking DMD exon 52 (DMD\u039452) exhibit molecular, clinical and pathological hallmarks of DMD, but die before sexual maturity and cannot be propagated by breeding. Therefore, we generated female DMD+\/\u2212 carriers. A single founder animal had 11 litters with 29 DMDY\/\u2212, 34 DMD+\/\u2212 as well as 36 male and 29 female wild-type offspring. Breeding with F1 and F2 DMD+\/\u2212 carriers resulted in an additional 114 DMDY\/\u2212 piglets. With intensive neonatal management, the majority survived for 3-4\u2005months, providing statistically relevant cohorts for experimental studies. Pathological investigations and proteome studies of skeletal muscles and myocardium confirmed the resemblance to human disease mechanisms. Importantly, DMDY\/\u2212 pigs displayed progressive myocardial fibrosis and increased expression of connexin-43, associated with significantly reduced left ventricular ejection fraction, at 3\u2005months. Furthermore, behavioral tests provided evidence for impaired cognitive ability. Our breeding cohort of DMD\u039452 pigs and standardized tissue repositories provide important resources for studying DMD disease mechanisms and for testing novel treatment strategies.<\/jats:p>","DOI":"10.1242\/dmm.049285","type":"journal-article","created":{"date-parts":[[2021,11,19]],"date-time":"2021-11-19T06:11:50Z","timestamp":1637302310000},"source":"Crossref","is-referenced-by-count":48,"title":["A scalable, clinically severe pig model for Duchenne muscular dystrophy"],"prefix":"10.1242","volume":"14","author":[{"given":"Michael","family":"Stirm","sequence":"first","affiliation":[{"name":"Chair for Molecular Animal Breeding and Biotechnology, Gene Center and Department of Veterinary Sciences, LMU Munich, 81377 Munich, Germany"},{"name":"Center for Innovative Medical Models (CiMM), LMU Munich, 85764 Oberschlei\u00dfheim, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lina Marie","family":"Fonteyne","sequence":"additional","affiliation":[{"name":"Chair 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