{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,29]],"date-time":"2026-10-29T09:31:20Z","timestamp":1793266280825,"version":"4.5.0"},"reference-count":52,"publisher":"SAGE Publications","issue":"6","license":[{"start":{"date-parts":[[2026,4,24]],"date-time":"2026-04-24T00:00:00Z","timestamp":1776988800000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by-nc\/4.0\/"},{"start":{"date-parts":[[2026,4,24]],"date-time":"2026-04-24T00:00:00Z","timestamp":1776988800000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/journals.sagepub.com\/page\/policies\/text-and-data-mining-license"}],"funder":[{"name":"Department of Defense, DMDRP","award":["#MD230014"],"award-info":[{"award-number":["#MD230014"]}]},{"DOI":"10.13039\/100005202","name":"Muscular Dystrophy Association","doi-asserted-by":"publisher","award":["MDA, research grant #MDA 957140"],"award-info":[{"award-number":["MDA, research grant #MDA 957140"]}],"id":[{"id":"10.13039\/100005202","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["journals.sagepub.com"],"crossmark-restriction":true},"short-container-title":["Journal of Neuromuscular Diseases"],"published-print":{"date-parts":[[2026,11]]},"abstract":"<jats:p>\n                    Muscle fibrosis is a key pathological feature of Duchenne muscular dystrophy (DMD) and is closely associated with disease progression. Fibroadipogenic progenitors (FAPs) are major contributors to fibrosis, yet the precise mechanisms remain unclear. To investigate FAP dynamics and lineage specification, we generated dual-reporter mice (PRURD2) by crossing D2.B10-Dmdmdx\/J (D2-mdx) mice with FAP and brown\/beige adipose tissue (BAT) reporter lines. Corresponding control mice (PRURDBA) were established on the DBA\/2J background. At 12 months, heart, diaphragm, and tibialis anterior (TA) muscles were collected for histological analysis. FAPs were isolated via FACS and subjected to single-cell RNA sequencing. PRURD2 mice exhibited increased fibrosis across all muscles compared to controls (\n                    <jats:italic toggle=\"yes\">p<\/jats:italic>\n                    \u2009&lt;\u20090.01) and a significant rise in PDGFR\u03b1-GFP\u2009+\u2009FAPs (\n                    <jats:italic toggle=\"yes\">p<\/jats:italic>\n                    \u2009&lt;\u20090.05). UMAP clustering identified 11 distinct FAP subpopulations, with the fibrosis-associated CD55 cluster enriched in PRURD2 mice. Pseudotime analysis showed lineage progression from progenitor clusters toward the fibrogenic CD55 cluster. CellChat analysis indicated increased interactions in PRURD2 mice involving fibrosis-related pathways like COLLAGEN, TGF-\u03b2, WNT, NOTCH, and ANGPTL. Additionally, fibrosis-related signaling pathways such as THY1, TWEAK, EPHA, EPHB, and SEMA6 showed increased interactions among FAP clusters in PRURD2 mice. Differential gene expression analysis revealed top upregulated genes including\n                    <jats:italic toggle=\"yes\">Cxcl13, Cxcl3, Ly6d, Klk1, Fgf23, Serpinb2, Mmp13, Ccl17<\/jats:italic>\n                    ,\n                    <jats:italic toggle=\"yes\">Postn<\/jats:italic>\n                    , and\n                    <jats:italic toggle=\"yes\">Adam12<\/jats:italic>\n                    . PRURD2 mice develop severe fibrosis in skeletal and cardiac muscle, driven by FAP-induced signaling pathways and genes. This model is valuable for understanding muscle fibrosis in DMD and developing anti-fibrotic therapies.\n                  <\/jats:p>","DOI":"10.1177\/22143602261441956","type":"journal-article","created":{"date-parts":[[2026,4,24]],"date-time":"2026-04-24T14:42:31Z","timestamp":1777041751000},"page":"1393-1405","update-policy":"https:\/\/doi.org\/10.1177\/sage-journals-update-policy","source":"Crossref","is-referenced-by-count":1,"title":["Fibrotic differentiation profile of skeletal and cardiac muscle fibroadipogenic progenitors in D2-mdx mouse"],"prefix":"10.1177","volume":"13","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-8322-463X","authenticated-orcid":false,"given":"Hiroyori","family":"Fusagawa","sequence":"first","affiliation":[{"name":"San Francisco Veterans Affairs Health Care System"},{"name":"Department of Orthopaedic Surgery, University of California, San Francisco, CA, USA"},{"name":"Division of Cellular Physiology and Signal Transduction in the Department of Physiology, Sapporo Medical University School of Medicine, Sapporo, Japan"},{"name":"Department of Orthopaedic Surgery, Sapporo Medical University School of Medicine, Sapporo, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-4904-1853","authenticated-orcid":false,"given":"Justin","family":"Lau","sequence":"additional","affiliation":[{"name":"San Francisco Veterans Affairs Health Care System"},{"name":"Department of Orthopaedic Surgery, University of California, San Francisco, CA, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0009-0002-6939-2703","authenticated-orcid":false,"given":"Sankalp","family":"Sharma","sequence":"additional","affiliation":[{"name":"San Francisco Veterans Affairs Health Care System"},{"name":"University of Minnesota"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mengyao","family":"Liu","sequence":"additional","affiliation":[{"name":"San Francisco Veterans Affairs Health Care System"},{"name":"Department of Orthopaedic Surgery, University of California, San Francisco, CA, USA"},{"name":"College of Medicine, California Northstate University, Elk Grove, CA, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0009-0003-1952-2511","authenticated-orcid":false,"given":"Yusef","family":"Samimi","sequence":"additional","affiliation":[{"name":"San Francisco Veterans Affairs Health Care System"},{"name":"Department of Orthopaedic Surgery, University of California, San Francisco, CA, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0009-0000-5487-916X","authenticated-orcid":false,"given":"Gabriel","family":"Franchet-Schaer","sequence":"additional","affiliation":[{"name":"San Francisco Veterans Affairs Health Care System"},{"name":"University of California, 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