{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,23]],"date-time":"2026-09-23T14:27:25Z","timestamp":1790173645315,"version":"4.1.0"},"reference-count":55,"publisher":"American Society for Clinical Investigation","issue":"11","funder":[{"name":"UCLA Center for Duchenne Muscular Dystrophy","award":["CureDuchenne Postdoctoral Fellowship"],"award-info":[{"award-number":["CureDuchenne Postdoctoral Fellowship"]}]},{"name":"AHA Scientist Development Grant","award":["AHA Scientist Development Grant"],"award-info":[{"award-number":["AHA Scientist Development Grant"]}]},{"name":"NIH T34","award":["GM008563"],"award-info":[{"award-number":["GM008563"]}]},{"name":"NIH R25","award":["GM050067"],"award-info":[{"award-number":["GM050067"]}]},{"name":"NIH","award":["HL128683"],"award-info":[{"award-number":["HL128683"]}]},{"name":"F31","award":["HL137408"],"award-info":[{"award-number":["HL137408"]}]},{"name":"NIH","award":["AR048179"],"award-info":[{"award-number":["AR048179"]}]},{"name":"NIH","award":["HL126204"],"award-info":[{"award-number":["HL126204"]}]},{"name":"NIH","award":["UL1TR000124"],"award-info":[{"award-number":["UL1TR000124"]}]},{"DOI":"10.13039\/100007683","name":"Nihon University","doi-asserted-by":"crossref","award":["5P30AR057230"],"award-info":[{"award-number":["5P30AR057230"]}],"id":[{"id":"10.13039\/100007683","id-type":"DOI","asserted-by":"crossref"}]},{"name":"MDA","award":["274143"],"award-info":[{"award-number":["274143"]}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2019,6,6]]},"abstract":"<jats:p>\n                    In the current preclinical study, we demonstrate the therapeutic potential of sarcospan (SSPN) overexpression to alleviate cardiomyopathy associated with Duchenne muscular dystrophy (DMD) utilizing dystrophin-deficient\n                    <jats:italic>mdx<\/jats:italic>\n                    mice with utrophin haploinsufficiency that more accurately represent the severe disease course of human DMD. SSPN interacts with dystrophin, the DMD disease gene product, and its autosomal paralog utrophin, which is upregulated in DMD as a partial compensatory mechanism. SSPN-Tg mice have enhanced abundance of fully glycosylated \u03b1-dystroglycan, which may further protect dystrophin-deficient cardiac membranes. Baseline echocardiography revealed that SSPN improves systolic function and hypertrophic indices in\n                    <jats:italic>mdx<\/jats:italic>\n                    and\n                    <jats:italic>mdx<\/jats:italic>\n                    :utr-heterozygous mice. Assessment of SSPN-Tg\n                    <jats:italic>mdx<\/jats:italic>\n                    mice by hemodynamic pressure-volume methods highlighted enhanced systolic performance compared with\n                    <jats:italic>mdx<\/jats:italic>\n                    controls. SSPN restored cardiac sarcolemma stability, the primary defect in DMD disease; reduced fibrotic response; and improved contractile function. We demonstrate that SSPN ameliorated more advanced cardiac disease in the context of diminished sarcolemma expression of utrophin and \u03b21D integrin, which mitigate disease severity, and partially restored responsiveness to \u03b2-adrenergic stimulation. Overall, our current and previous findings suggest that SSPN overexpression in DMD mouse models positively affects skeletal, pulmonary, and cardiac performance by addressing the stability of proteins at the sarcolemma that protect the heart from injury, supporting SSPN and membrane stabilization as a therapeutic target for DMD.\n                  <\/jats:p>","DOI":"10.1172\/jci.insight.123855","type":"journal-article","created":{"date-parts":[[2019,4,30]],"date-time":"2019-04-30T12:45:43Z","timestamp":1556628343000},"source":"Crossref","is-referenced-by-count":28,"title":["Stabilization of the cardiac sarcolemma by sarcospan rescues DMD-associated cardiomyopathy"],"prefix":"10.1172","volume":"4","author":[{"given":"Michelle S.","family":"Parvatiyar","sequence":"first","affiliation":[{"name":"Department of Integrative Biology & Physiology and"},{"name":"Center for Duchenne Muscular Dystrophy, UCLA, Los Angeles, California, 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