{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,30]],"date-time":"2026-09-30T19:15:26Z","timestamp":1790795726188,"version":"4.1.0"},"reference-count":23,"publisher":"Oxford University Press (OUP)","issue":"13","license":[{"start":{"date-parts":[[2020,5,29]],"date-time":"2020-05-29T00:00:00Z","timestamp":1590710400000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/academic.oup.com\/journals\/pages\/open_access\/funder_policies\/chorus\/standard_publication_model"}],"funder":[{"DOI":"10.13039\/100000002","name":"National Institutes of Health","doi-asserted-by":"publisher","award":["MDA238981"],"award-info":[{"award-number":["MDA238981"]}],"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":["MDA628561"],"award-info":[{"award-number":["MDA628561"]}],"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":["R01AR064338-01A1"],"award-info":[{"award-number":["R01AR064338-01A1"]}],"id":[{"id":"10.13039\/100000002","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2020,8,3]]},"abstract":"<jats:title>Abstract<\/jats:title>\n               <jats:p>Laminin-\u03b12 related congenital muscular dystrophy (LAMA2-CMD) is a fatal muscle disease caused by mutations in the LAMA2 gene. Laminin-\u03b12 is critical for the formation of laminin-211 and -221 heterotrimers in the muscle basal lamina. LAMA2-CMD patients exhibit hypotonia from birth and progressive muscle loss that results in developmental delay, confinement to a wheelchair, respiratory insufficiency and premature death. There is currently no cure or effective treatment for LAMA2-CMD. Several studies have shown laminin-111 can serve as an effective protein-replacement therapy for LAMA2-CMD. Studies have demonstrated early treatment with laminin-111 protein results in an increase in life expectancy and improvements in muscle pathology and function. Since LAMA2-CMD patients are often diagnosed after advanced disease, it is unclear if laminin-111 protein therapy at an advanced stage of the disease can have beneficial outcomes. In this study, we tested the efficacy of laminin-111 protein therapy after disease onset in a mouse model of LAMA2-CMD. Our results showed laminin-111 treatment after muscle disease onset increased life expectancy, promoted muscle growth and increased muscle stiffness. Together these studies indicate laminin-111 protein therapy either early or late in the disease process could serve as an effective protein replacement therapy for LAMA2-CMD.<\/jats:p>","DOI":"10.1093\/hmg\/ddaa104","type":"journal-article","created":{"date-parts":[[2020,5,28]],"date-time":"2020-05-28T19:09:43Z","timestamp":1590692983000},"page":"2162-2170","source":"Crossref","is-referenced-by-count":8,"title":["Laminin-111 protein therapy after disease onset slows muscle disease in a mouse model of laminin-\u03b12 related congenital muscular dystrophy"],"prefix":"10.1093","volume":"29","author":[{"given":"Pamela","family":"Barraza-Flores","sequence":"first","affiliation":[{"name":"Department of Pharmacology, Reno School of Medicine, University of Nevada, Reno, NV 89557, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Katherine E","family":"Bukovec","sequence":"additional","affiliation":[{"name":"Department of Human Nutrition, Foods, and Exercise, Virginia Polytechnic Institute and State University, Blacksburg, VA 24061, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marisela","family":"Dagda","sequence":"additional","affiliation":[{"name":"Department of Pharmacology, Reno School of Medicine, University of Nevada, Reno, NV 89557, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Brandon W","family":"Conner","sequence":"additional","affiliation":[{"name":"Department of Pharmacology, Reno School of Medicine, University of Nevada, Reno, NV 89557, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ariany","family":"Oliveira-Santos","sequence":"additional","affiliation":[{"name":"Department of Pharmacology, Reno School of Medicine, University of Nevada, Reno, NV 89557, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Robert W","family":"Grange","sequence":"additional","affiliation":[{"name":"Department of Human Nutrition, Foods, and Exercise, Virginia Polytechnic Institute and State University, Blacksburg, VA 24061, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Dean J","family":"Burkin","sequence":"additional","affiliation":[{"name":"Department of Pharmacology, Reno School of Medicine, University of Nevada, Reno, NV 89557, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"286","published-online":{"date-parts":[[2020,5,29]]},"reference":[{"key":"2020091713241409200_ref1","doi-asserted-by":"crossref","first-page":"904","DOI":"10.1212\/WNL.0000000000001303","article-title":"Prevalence of congenital muscular dystrophy in Italy","volume":"84","author":"Graziano","year":"2015","journal-title":"Neurology"},{"key":"2020091713241409200_ref2","doi-asserted-by":"crossref","first-page":"844","DOI":"10.1172\/JCI3705","article-title":"Merosin-deficient congenital muscular dystrophy. 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