{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,8]],"date-time":"2026-10-08T20:50:08Z","timestamp":1791492608636,"version":"4.3.4"},"reference-count":85,"publisher":"American Society for Clinical Investigation","issue":"22","license":[{"start":{"date-parts":[[2025,11,24]],"date-time":"2025-11-24T00:00:00Z","timestamp":1763942400000},"content-version":"vor","delay-in-days":39,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"DOI":"10.13039\/100000065","name":"NINDS","doi-asserted-by":"crossref","award":["1R01NS136281-01"],"award-info":[{"award-number":["1R01NS136281-01"]}],"id":[{"id":"10.13039\/100000065","id-type":"DOI","asserted-by":"crossref"}]},{"DOI":"10.13039\/100000065","name":"NINDS","doi-asserted-by":"crossref","award":["P50NS053672"],"award-info":[{"award-number":["P50NS053672"]}],"id":[{"id":"10.13039\/100000065","id-type":"DOI","asserted-by":"crossref"}]},{"name":"CureCMD","award":["1018515"],"award-info":[{"award-number":["1018515"]}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2025,11,24]]},"abstract":"<jats:p>\n                    Laminin-\u03b12\u2013related congenital muscular dystrophy (LAMA2-CMD) is a severe neuromuscular disorder caused by mutations in the\n                    <jats:italic>LAMA2<\/jats:italic>\n                    gene, leading to loss of heterotrimers laminin-211\/221, key components of the skeletal muscle extracellular matrix. Their absence disrupts adhesion between the cytoskeleton and extracellular matrix, resulting in progressive muscle wasting. Laminin-211\/221 interacts with adhesion complexes such as the dystrophin\/utrophin glycoprotein complex and \u03b17\u03b21-integrin. However, the regulatory mechanisms of these laminin-binding complexes and the broader role of laminin\u2019s influence on the formation of the macromolecular network in skeletal muscle remain unclear. We previously demonstrated that delivering mouse laminin-111 to the\n                    <jats:italic>\n                      dy\n                      <jats:sup>W\u2013\/\u2013<\/jats:sup>\n                    <\/jats:italic>\n                    mouse model of LAMA2-CMD prevented disease progression, improved strength, and extended survival. We hypothesize that laminin-111, the embryonic laminin isoform, restores key adhesion-signaling networks. Using spatial proteomics on patient and mouse muscle, we identified loss of essential signaling components: heat shock proteins 27 and 70, c-Jun N-terminal kinase, and glucose transporter 1 in laminin-\u03b12\u2013deficient muscle. Treatment with recombinant human laminin-111 (rhLAM-111) restored protein localization, reduced ROS, and promoted glycolytic, prosurvival signaling. These findings highlight laminin\u2019s role in maintaining muscle homeostasis and metabolism and support the therapeutic potential of rhLAM-111 for treating LAMA2-CMD by restoring adhesion and intracellular signaling in dystrophic muscle.\n                  <\/jats:p>","DOI":"10.1172\/jci.insight.194581","type":"journal-article","created":{"date-parts":[[2025,10,21]],"date-time":"2025-10-21T17:32:11Z","timestamp":1761067931000},"source":"Crossref","is-referenced-by-count":2,"title":["Spatial proteomics reveals recombinant human laminin-111 restores adhesion signaling to laminin-\u03b12\u2013deficient muscle"],"prefix":"10.1172","volume":"10","author":[{"given":"Hailey J.","family":"Hermann","sequence":"first","affiliation":[{"name":"Department of Pharmacology, Reno School of Medicine, University of Nevada, Reno, Nevada, USA."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ryan D.","family":"Wuebbles","sequence":"additional","affiliation":[{"name":"Department of 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