{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,2,13]],"date-time":"2026-02-13T21:15:17Z","timestamp":1771017317360,"version":"3.50.1"},"reference-count":36,"publisher":"American Physiological Society","issue":"1","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Journal of Applied Physiology"],"published-print":{"date-parts":[[2011,7]]},"abstract":"<jats:p> Previously, we demonstrated functional substitution of dystrophin by TAT-\u03bcUtrophin (TAT-\u03bcUtr) in dystrophin-deficient mdx mice. Herein, we addressed whether TAT-\u03bcUtr could improve the phenotype of dystrophin and utrophin double-knockout ( mdx:utr<jats:sup>\u2212\/\u2212<\/jats:sup>) mice. Specifically, we quantitatively compared survival and quality of life assessments in mdx:utr<jats:sup>\u2212\/\u2212<\/jats:sup> mice receiving TAT-\u03bcUtr protein administration against placebo-treated mdx:utr<jats:sup>\u2212\/\u2212<\/jats:sup> mice (PBS). Additionally, skeletal muscles from TAT-\u03bcUtr and PBS mice were tested in vivo and ex vivo for strength and susceptibility to eccentric contraction-induced injury. We found the TAT-\u03bcUtr treatment extended life span 45% compared with mice administered PBS. This was attributed to significantly increased food consumption (3.1 vs. 1.8 g\/24 h) due to improved ability to search for food as daily cage activities were greater in TAT-\u03bcUtr mice (e.g., 364 vs. 201 m ambulation\/24 h). The extensor digitorum longus muscles of TAT-\u03bcUtr-treated double-knockout mice also displayed increased force-generating capacity ex vivo (8.3 vs. 6.4 N\/cm<jats:sup>2<\/jats:sup>) and decreased susceptibility to injury ex vivo and in vivo. These data indicate that the functional benefits of TAT-\u03bcUtr replacement treatment extend to the mdx:utr<jats:sup>\u2212\/\u2212<\/jats:sup> double-knockout mouse and support its development as a therapy to mitigate muscle weakness in patients with Duchenne muscular dystrophy. <\/jats:p>","DOI":"10.1152\/japplphysiol.00248.2011","type":"journal-article","created":{"date-parts":[[2011,5,13]],"date-time":"2011-05-13T02:27:59Z","timestamp":1305253679000},"page":"200-205","source":"Crossref","is-referenced-by-count":17,"title":["TAT-\u03bcUtrophin mitigates the pathophysiology of dystrophin and utrophin double-knockout mice"],"prefix":"10.1152","volume":"111","author":[{"given":"Jarrod A.","family":"Call","sequence":"first","affiliation":[{"name":"Program in Physical Therapy and Rehabilitation Sciences,"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"James M.","family":"Ervasti","sequence":"additional","affiliation":[{"name":"Department of Biochemistry, Molecular 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