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A number of animal models of muscular dystrophy exist, but the most effective model for characterizing the structural and functional properties of dystrophin and therapeutic interventions has been the\n                    <jats:italic>mdx<\/jats:italic>\n                    mouse. Despite the ~20 years of investigations of the\n                    <jats:italic>mdx<\/jats:italic>\n                    mouse, the impact of the disease on the life span of\n                    <jats:italic>mdx<\/jats:italic>\n                    mice and the cause of death remain unresolved. Consequently, a life span study of the\n                    <jats:italic>mdx<\/jats:italic>\n                    mouse was designed that included cohorts of male and female\n                    <jats:italic>mdx<\/jats:italic>\n                    and wild\u2010type C57BL\/10 mice housed under specific pathogen\u2010free conditions with deaths restricted to natural causes and with examination of the carcasses for pathology. Compared with wild\u2010type mice, both\n                    <jats:italic>mdx<\/jats:italic>\n                    male and female mice had reduced life spans and displayed a progressively dystrophic muscle histopa\u2010thology. Surprisingly, old\n                    <jats:italic>mdx<\/jats:italic>\n                    mice were prone to develop muscle tumors that resembled the human form of alveolar rhabdomyosarcoma, a cancer associated with poor prognosis. Rhabdomyosarcomas have not been observed previously in nontransgenic mice. The results substantiate the\n                    <jats:italic>mdx<\/jats:italic>\n                    mouse as an important model system for studies of the pathogenesis of and potential remedies for DMD.\u2013Chamberlain, J. S., Metzger, J., Reyes, M., Townsend, D., Faulkner, J. A. Dystrophin\u2010deficient\n                    <jats:italic>mdx<\/jats:italic>\n                    mice display a reduced life span and are susceptible to spontaneous rhabdomyo\u2010sarcoma.\n                    <jats:italic>FASEB J.<\/jats:italic>\n                    21, 2195\u20132204 (2007)\n                  <\/jats:p>","DOI":"10.1096\/fj.06-7353com","type":"journal-article","created":{"date-parts":[[2007,3,14]],"date-time":"2007-03-14T20:36:25Z","timestamp":1173904585000},"page":"2195-2204","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":314,"title":["Dystrophin\u2010deficient\n                    <i>mdx<\/i>\n                    mice display a reduced life span and are susceptible to spontaneous rhabdomyosarcoma"],"prefix":"10.1096","volume":"21","author":[{"given":"Jeffrey S.","family":"Chamberlain","sequence":"first","affiliation":[{"name":"Department of Neurology University of Washington School of Medicine Seattle Washington USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Joseph","family":"Metzger","sequence":"additional","affiliation":[{"name":"Department of Molecular and Integrative Physiology Institute of Gerontology University of Michigan Ann Arbor Michigan USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Morayma","family":"Reyes","sequence":"additional","affiliation":[{"name":"Department of Neurology University of Washington School of Medicine Seattle Washington USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"DeWayne","family":"Townsend","sequence":"additional","affiliation":[{"name":"Department of Molecular and Integrative Physiology Institute of Gerontology University of Michigan Ann Arbor Michigan USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"John A.","family":"Faulkner","sequence":"additional","affiliation":[{"name":"Department of Molecular and Integrative Physiology Institute of Gerontology University of Michigan Ann Arbor Michigan USA"},{"name":"Institute of Gerontology University of Michigan Ann Arbor Michigan USA"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2007,3,14]]},"reference":[{"key":"e_1_2_6_2_1","first-page":"41","volume-title":"Current Clinical Practices, series","author":"Moser H.","year":"1984"},{"key":"e_1_2_6_3_1","doi-asserted-by":"publisher","DOI":"10.1177\/095952875203500108"},{"key":"e_1_2_6_4_1","first-page":"5","article-title":"Recherches sur la paralysie muscu-laire pseudohypertrophique ou paralysie myo-sclerosique","volume":"11","author":"Duchenne G. 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