{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,31]],"date-time":"2026-10-31T16:29:37Z","timestamp":1793464177981,"version":"4.5.0"},"reference-count":76,"publisher":"Springer Science and Business Media LLC","issue":"12","license":[{"start":{"date-parts":[[2023,11,6]],"date-time":"2023-11-06T00:00:00Z","timestamp":1699228800000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"},{"start":{"date-parts":[[2023,11,6]],"date-time":"2023-11-06T00:00:00Z","timestamp":1699228800000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"name":"Association Fran\u00e7aise contre les Myopathies","award":["23758"],"award-info":[{"award-number":["23758"]}]},{"name":"GFB\u2010ONLUS","award":["PR\u20100394"],"award-info":[{"award-number":["PR\u20100394"]}]},{"DOI":"10.13039\/501100003196","name":"Ministero della Salute","doi-asserted-by":"publisher","award":["RF\u20102016\u201002362263"],"award-info":[{"award-number":["RF\u20102016\u201002362263"]}],"id":[{"id":"10.13039\/501100003196","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100005202","name":"Muscular Dystrophy Association","doi-asserted-by":"publisher","award":["874294"],"award-info":[{"award-number":["874294"]}],"id":[{"id":"10.13039\/100005202","id-type":"DOI","asserted-by":"publisher"}]},{"name":"POR FESR Regione Lombardia","award":["2526393"],"award-info":[{"award-number":["2526393"]}]},{"name":"Fondazione Telethon Provincia Autonoma di Trento \u2013 Italy","award":["TCP13007"],"award-info":[{"award-number":["TCP13007"]}]},{"DOI":"10.13039\/501100008530","name":"European Regional Development Fund","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100008530","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["link.springer.com"],"crossmark-restriction":false},"short-container-title":["EMBO Mol Med"],"published-print":{"date-parts":[[2023,12,7]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:p>Fibrosis is associated with compromised muscle functionality in Duchenne muscular dystrophy (DMD). We report observations with tissues from dystrophic patients and mice supporting a model to explain fibrosis in DMD, which relies on the crosstalk between the complement and the WNT signaling pathways and the functional interactions of two cellular types. Fibro\u2010adipogenic progenitors and macrophages, which populate the inflamed dystrophic muscles, act as a combinatorial source of WNT activity by secreting distinct subunits of the C1 complement complex. The resulting aberrant activation of the WNT signaling in responsive cells, such as fibro\u2010adipogenic progenitors, contributes to fibrosis. Indeed, pharmacological inhibition of the C1r\/s subunits in a murine model of DMD mitigated the activation of the WNT signaling pathway, reduced the fibrogenic characteristics of the fibro\u2010adipogenic progenitors, and ameliorated the dystrophic phenotype. These studies shed new light on the molecular and cellular mechanisms responsible for fibrosis in muscular dystrophy and open to new therapeutic strategies.<\/jats:p>","DOI":"10.15252\/emmm.202317405","type":"journal-article","created":{"date-parts":[[2023,11,6]],"date-time":"2023-11-06T03:50:22Z","timestamp":1699242622000},"update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":11,"title":["Combinatorial activation of the WNT\u2010dependent fibrogenic program by distinct complement subunits in dystrophic muscle"],"prefix":"10.1038","volume":"15","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-8183-5226","authenticated-orcid":false,"given":"Francesca","family":"Florio","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sara","family":"Vencato","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-7821-8198","authenticated-orcid":false,"given":"Filomena T","family":"Papa","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-4329-3221","authenticated-orcid":false,"given":"Michela","family":"Libergoli","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-5646-8751","authenticated-orcid":false,"given":"Eyemen","family":"Kheir","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-8181-9445","authenticated-orcid":false,"given":"Imen","family":"Ghzaiel","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Thomas A","family":"Rando","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-2705-3984","authenticated-orcid":false,"given":"Yvan","family":"Torrente","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-8631-3419","authenticated-orcid":false,"given":"Stefano","family":"Biressi","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2023,11,6]]},"reference":[{"key":"BFEMMM202317405_CR1","doi-asserted-by":"crossref","first-page":"735","DOI":"10.1038\/ncomms1734","volume":"3","author":"A Akhmetshina","year":"2012","unstructured":"Akhmetshina A, Palumbo K, Dees C, Bergmann C, Venalis P, Zerr P, Horn A, Kireva T, Beyer C, Zwerina J et\u00a0al (2012) Activation of canonical Wnt signalling is required for TGF\u2010\u03b2\u2010mediated fibrosis. Nat Commun 3: 735","journal-title":"Nat Commun"},{"key":"BFEMMM202317405_CR2","doi-asserted-by":"crossref","DOI":"10.1126\/scitranslmed.abg7504","volume":"14","author":"F Babaeijandaghi","year":"2022","unstructured":"Babaeijandaghi F, Cheng R, Kajabadi N, Soliman H, Chang CK, Smandych J, Tung LW, Long R, Ghassemi A, Rossi FMV (2022) Metabolic reprogramming of skeletal muscle by resident macrophages points to CSF1R inhibitors as muscular dystrophy therapeutics. Sci Transl Med 14: eabg7504","journal-title":"Sci Transl Med"},{"key":"BFEMMM202317405_CR3","doi-asserted-by":"crossref","first-page":"195","DOI":"10.1016\/j.ydbio.2013.04.021","volume":"379","author":"S Biressi","year":"2013","unstructured":"Biressi S, Bjornson CRR, Carlig PMM, Nishijo K, Keller C, Rando TA (2013) Myf5 expression during fetal myogenesis defines the developmental progenitors of adult satellite cells. Dev Biol 379: 195\u2013207","journal-title":"Dev Biol"},{"key":"BFEMMM202317405_CR4","doi-asserted-by":"crossref","DOI":"10.1126\/scitranslmed.3008411","volume":"6","author":"S Biressi","year":"2014","unstructured":"Biressi S, Miyabara EH, Gopinath SD, Carlig PMM, Rando TA (2014) A Wnt\u2010TGF\u03b22 axis induces a fibrogenic program in muscle stem cells from dystrophic mice. Sci Transl Med 6: 267ra176","journal-title":"Sci Transl Med"},{"key":"BFEMMM202317405_CR5","doi-asserted-by":"crossref","first-page":"5652","DOI":"10.1172\/JCI142031","volume":"130","author":"S Biressi","year":"2020","unstructured":"Biressi S, Filareto A, Rando TA (2020) Stem cell therapy for muscular dystrophies. J Clin Invest 130: 5652\u20135664","journal-title":"J Clin Invest"},{"key":"BFEMMM202317405_CR6","doi-asserted-by":"crossref","first-page":"251","DOI":"10.1016\/S1474-4422(18)30024-3","volume":"17","author":"DJ Birnkrant","year":"2018","unstructured":"Birnkrant DJ, Bushby K, Bann CM, Apkon SD, Blackwell A, Brumbaugh D, Case LE, Clemens PR, Hadjiyannakis S, Pandya S et\u00a0al (2018) Diagnosis and management of Duchenne muscular dystrophy, part 1: diagnosis, and neuromuscular, rehabilitation, endocrine, and gastrointestinal and nutritional management. Lancet Neurol 17: 251\u2013267","journal-title":"Lancet Neurol"},{"key":"BFEMMM202317405_CR7","first-page":"1413","volume":"38","author":"P Blyszczuk","year":"2017","unstructured":"Blyszczuk P, M\u00fcller\u2010Edenborn B, Valenta T, Osto E, Stellato M, Behnke S, Glatz K, Basler K, L\u00fcscher TF, Distler O et\u00a0al (2017) Transforming growth factor\u2010\u03b2\u2010dependent Wnt secretion controls myofibroblast formation and myocardial fibrosis progression in experimental autoimmune myocarditis. Eur Heart J 38: 1413\u20131425","journal-title":"Eur Heart J"},{"key":"BFEMMM202317405_CR8","doi-asserted-by":"crossref","first-page":"807","DOI":"10.1126\/science.1144090","volume":"317","author":"AS Brack","year":"2007","unstructured":"Brack AS, Conboy MJ, Roy S, Lee M, Kuo CJ, Keller C, Rando TA (2007) Increased Wnt signaling during aging alters muscle stem cell fate and increases fibrosis. Science 317: 807\u2013810","journal-title":"Science"},{"key":"BFEMMM202317405_CR9","doi-asserted-by":"crossref","first-page":"1282","DOI":"10.1016\/j.cell.2013.10.054","volume":"155","author":"D Burzyn","year":"2013","unstructured":"Burzyn D, Kuswanto W, Kolodin D, Shadrach JL, Cerletti M, Jang Y, Sefik E, Tan TG, Wagers AJ, Benoist C et\u00a0al (2013) A special population of regulatory T cells potentiates muscle repair. Cell 155: 1282\u20131295","journal-title":"Cell"},{"key":"BFEMMM202317405_CR10","doi-asserted-by":"crossref","DOI":"10.1371\/journal.pone.0019809","volume":"6","author":"JM Carthy","year":"2011","unstructured":"Carthy JM, Garmaroudi FS, Luo Z, McManus BM (2011) Wnt3a induces myofibroblast differentiation by upregulating TGF\u2010\u03b2 signaling through SMAD2 in a \u03b2\u2010Catenin\u2010dependent manner. PLoS ONE 6: e19809","journal-title":"PLoS ONE"},{"key":"BFEMMM202317405_CR11","doi-asserted-by":"crossref","first-page":"785","DOI":"10.1038\/nrc2696","volume":"9","author":"HZ Chen","year":"2009","unstructured":"Chen HZ, Tsai SY, Leone G (2009) Emerging roles of E2Fs in cancer: an exit from cell cycle control. Nat Rev Cancer 9: 785\u2013797","journal-title":"Nat Rev Cancer"},{"key":"BFEMMM202317405_CR12","doi-asserted-by":"crossref","first-page":"906","DOI":"10.2174\/138161210790883453","volume":"16","author":"S Ciciliot","year":"2010","unstructured":"Ciciliot S, Schiaffino S (2010) Regeneration of mammalian skeletal muscle. Basic mechanisms and clinical implications. Curr Pharm Des 16: 906\u2013914","journal-title":"Curr Pharm Des"},{"key":"BFEMMM202317405_CR13","doi-asserted-by":"crossref","first-page":"510","DOI":"10.2174\/1566524014666140414210346","volume":"14","author":"P Cisternas","year":"2014","unstructured":"Cisternas P, Vio CP, Inestrosa NC (2014) Role of Wnt signaling in tissue fibrosis, lessons from skeletal muscle and kidney. Curr Mol Med 14: 510\u2013522","journal-title":"Curr Mol Med"},{"key":"BFEMMM202317405_CR14","doi-asserted-by":"crossref","first-page":"647","DOI":"10.1007\/s00441-015-2343-0","volume":"364","author":"O Contreras","year":"2016","unstructured":"Contreras O, Rebolledo DL, Oyarz\u00fan JE, Olgu\u00edn HC, Brandan E (2016) Connective tissue cells expressing fibro\/adipogenic progenitor markers increase under chronic damage: relevance in fibroblast\u2010myofibroblast differentiation and skeletal muscle fibrosis. Cell Tissue Res 364: 647\u2013660","journal-title":"Cell Tissue Res"},{"key":"BFEMMM202317405_CR15","doi-asserted-by":"crossref","first-page":"E8996","DOI":"10.1073\/pnas.1708725114","volume":"114","author":"A de Morr\u00e9e","year":"2017","unstructured":"de Morr\u00e9e A, van Velthoven CTJ, Gan Q, Salvi JS, Klein JDD, Akimenko I, Quarta M, Biressi S, Rando TA (2017) Staufen1 inhibits MyoD translation to actively maintain muscle stem cell quiescence. Proc Natl Acad Sci USA 114: E8996\u2013E9005","journal-title":"Proc Natl Acad Sci USA"},{"key":"BFEMMM202317405_CR16","doi-asserted-by":"crossref","first-page":"762","DOI":"10.1097\/NEN.0b013e3181aa31c2","volume":"68","author":"I Desguerre","year":"2009","unstructured":"Desguerre I, Mayer M, Leturcq F, Barbet J\u2010P, Gherardi RK, Christov C (2009) Endomysial fibrosis in Duchenne muscular dystrophy: a marker of poor outcome associated with macrophage alternative activation. J Neuropathol Exp Neurol 68: 762\u2013773","journal-title":"J Neuropathol Exp Neurol"},{"key":"BFEMMM202317405_CR17","doi-asserted-by":"crossref","first-page":"803","DOI":"10.1002\/mus.23341","volume":"45","author":"I Desguerre","year":"2012","unstructured":"Desguerre I, Arnold L, Vignaud A, Cuvellier S, Yacoub\u2010Youssef H, Gherardi RK, Chelly J, Chretien F, Mounier R, Ferry A et\u00a0al (2012) A new model of experimental fibrosis in hindlimb skeletal muscle of adult mdx mouse mimicking muscular dystrophy. Muscle Nerve 45: 803\u2013814","journal-title":"Muscle Nerve"},{"key":"BFEMMM202317405_CR18","doi-asserted-by":"crossref","first-page":"1262","DOI":"10.1038\/nm.2848","volume":"18","author":"S Dulauroy","year":"2012","unstructured":"Dulauroy S, Di Carlo SE, Langa F, Eberl G, Peduto L (2012) Lineage tracing and genetic ablation of ADAM12(+) perivascular cells identify a major source of profibrotic cells during acute tissue injury. Nat Med 18: 1262\u20131270","journal-title":"Nat Med"},{"key":"BFEMMM202317405_CR19","doi-asserted-by":"crossref","first-page":"687","DOI":"10.1016\/S0140-6736(02)07815-7","volume":"359","author":"AEH Emery","year":"2002","unstructured":"Emery AEH (2002) The muscular dystrophies. Lancet 359: 687\u2013695","journal-title":"Lancet"},{"key":"BFEMMM202317405_CR20","doi-asserted-by":"crossref","first-page":"289","DOI":"10.1002\/ana.410120314","volume":"12","author":"AG Engel","year":"1982","unstructured":"Engel AG, Biesecker G (1982) Complement activation in muscle fiber necrosis: demonstration of the membrane attack complex of complement in necrotic fibers. Ann Neurol 12: 289\u2013296","journal-title":"Ann Neurol"},{"key":"BFEMMM202317405_CR21","doi-asserted-by":"crossref","first-page":"117","DOI":"10.1038\/nri3800","volume":"15","author":"S Epelman","year":"2015","unstructured":"Epelman S, Liu PP, Mann DL (2015) Role of innate and adaptive immune mechanisms in cardiac injury and repair. Nat Rev Immunol 15: 117\u2013129","journal-title":"Nat Rev Immunol"},{"key":"BFEMMM202317405_CR22","doi-asserted-by":"crossref","first-page":"2201","DOI":"10.1016\/j.cmet.2021.10.001","volume":"33","author":"J Farup","year":"2021","unstructured":"Farup J, Just J, de Paoli F, Lin L, Jensen JB, Billeskov T, Roman IS, C\u00f6mert C, M\u00f8ller AB, Madaro L et\u00a0al (2021) Human skeletal muscle CD90 + fibro\u2010adipogenic progenitors are associated with muscle degeneration in type 2 diabetic patients. Cell Metab 33: 2201\u20132214","journal-title":"Cell Metab"},{"key":"BFEMMM202317405_CR23","doi-asserted-by":"crossref","first-page":"2566","DOI":"10.1111\/trf.12678","volume":"54","author":"A Feussner","year":"2014","unstructured":"Feussner A, Kalina U, Hofmann P, Machnig T, Henkel G (2014) Biochemical comparison of four commercially available C1 esterase inhibitor concentrates for treatment of hereditary angioedema. Transfusion 54: 2566\u20132573","journal-title":"Transfusion"},{"key":"BFEMMM202317405_CR24","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1159\/000068446","volume":"99","author":"J Finsterer","year":"2003","unstructured":"Finsterer J, St\u00f6llberger C (2003) The heart in human dystrophinopathies. Cardiology 99: 1\u201319","journal-title":"Cardiology"},{"key":"BFEMMM202317405_CR25","doi-asserted-by":"crossref","DOI":"10.3389\/fphys.2022.834705","volume":"13","author":"F Florio","year":"2022","unstructured":"Florio F, Accordini S, Libergoli M, Biressi S (2022) Targeting muscle\u2010resident single cells through in vivo electro\u2010enhanced plasmid transfer in healthy and compromised skeletal muscle. Front Physiol 13: 834705","journal-title":"Front Physiol"},{"key":"BFEMMM202317405_CR26","doi-asserted-by":"crossref","first-page":"152","DOI":"10.1001\/archneur.1981.00510030046006","volume":"38","author":"M Foidart","year":"1981","unstructured":"Foidart M, Foidart JM, Engel WK (1981) Collagen localization in normal and fibrotic human skeletal muscle. Arch Neurol 38: 152\u2013157","journal-title":"Arch Neurol"},{"key":"BFEMMM202317405_CR27","doi-asserted-by":"crossref","first-page":"18549","DOI":"10.1073\/pnas.1311628110","volume":"110","author":"RM George","year":"2013","unstructured":"George RM, Biressi S, Beres BJ, Rogers E, Mulia AK, Allen RE, Rawls A, Rando TA, Wilson\u2010Rawls J (2013) Numb\u2010deficient satellite cells have regeneration and proliferation defects. Proc Natl Acad Sci USA 110: 18549\u201318554","journal-title":"Proc Natl Acad Sci USA"},{"key":"BFEMMM202317405_CR28","doi-asserted-by":"crossref","first-page":"157","DOI":"10.1016\/bs.pmbts.2017.11.026","volume":"153","author":"F Girardi","year":"2018","unstructured":"Girardi F, Le Grand F (2018) Wnt signaling in skeletal muscle development and regeneration. Prog Mol Biol Transl Sci 153: 157\u2013179","journal-title":"Prog Mol Biol Transl Sci"},{"key":"BFEMMM202317405_CR29","doi-asserted-by":"crossref","first-page":"13","DOI":"10.1007\/BF00302666","volume":"14","author":"P Gulati","year":"1994","unstructured":"Gulati P, Lemercier C, Lappin D, Whaley K, Gue D (1994) Expression of the components and regulatory proteins of the classical pathway of complement in normal and diseased synovium. Rheumatol Int 14: 13\u201319","journal-title":"Rheumatol Int"},{"key":"BFEMMM202317405_CR30","doi-asserted-by":"crossref","first-page":"376","DOI":"10.1016\/j.cell.2013.02.053","volume":"153","author":"JE Heredia","year":"2013","unstructured":"Heredia JE, Mukundan L, Chen FM, Mueller AA, Deo RC, Locksley RM, Rando TA, Chawla A (2013) Type 2 innate signals stimulate fibro\/adipogenic progenitors to facilitate muscle regeneration. Cell 153: 376\u2013388","journal-title":"Cell"},{"key":"BFEMMM202317405_CR31","doi-asserted-by":"crossref","first-page":"410","DOI":"10.1002\/path.4801","volume":"240","author":"N Ieronimakis","year":"2016","unstructured":"Ieronimakis N, Hays A, Prasad A, Janebodin K, Duffield JS, Reyes M (2016) PDGFR\u03b1 signalling promotes fibrogenic responses in collagen\u2010producing cells in Duchenne muscular dystrophy. J Pathol 240: 410\u2013424","journal-title":"J Pathol"},{"key":"BFEMMM202317405_CR32","doi-asserted-by":"crossref","first-page":"893","DOI":"10.1182\/blood-2018-06-856930","volume":"133","author":"U J\u00e4ger","year":"2019","unstructured":"J\u00e4ger U, D'Sa S, Sch\u00f6rgenhofer C, Bartko J, Derhaschnig U, Sillaber C, Jilma\u2010Stohlawetz P, Fillitz M, Schenk T, Patou G et\u00a0al (2019) Inhibition of complement C1s improves severe hemolytic anemia in cold agglutinin disease: a first\u2010in\u2010human trial. Blood 133: 893\u2013901","journal-title":"Blood"},{"key":"BFEMMM202317405_CR33","doi-asserted-by":"crossref","first-page":"153","DOI":"10.1038\/ncb2015","volume":"12","author":"AWB Joe","year":"2010","unstructured":"Joe AWB, Yi L, Natarajan A, Le Grand F, So L, Wang J, Rudnicki MA, Rossi FMV (2010) Muscle injury activates resident fibro\/adipogenic progenitors that facilitate myogenesis. Nat Cell Biol 12: 153\u2013163","journal-title":"Nat Cell Biol"},{"key":"BFEMMM202317405_CR34","volume":"376","author":"RC Jones","year":"2022","unstructured":"Jones RC, Karkanias J, Krasnow MA, Pisco AO, Quake SR, Salzman J, Yosef N, Bulthaup B, Brown P, Harper W et\u00a0al (2022) The Tabula Sapiens: a multiple\u2010organ, single\u2010cell transcriptomic atlas of humans. Science 376: eabl4896","journal-title":"Science"},{"key":"BFEMMM202317405_CR35","doi-asserted-by":"crossref","first-page":"93","DOI":"10.1007\/978-1-4939-7283-8_7","volume":"1668","author":"RN Judson","year":"2017","unstructured":"Judson RN, Low M, Eisner C, Rossi FM (2017) Isolation, culture, and differentiation of Fibro\/Adipogenic Progenitors (FAPs) from skeletal muscle. Methods Mol Biol 1668: 93\u2013103","journal-title":"Methods Mol Biol"},{"key":"BFEMMM202317405_CR36","doi-asserted-by":"crossref","first-page":"7307","DOI":"10.1073\/pnas.85.19.7307","volume":"85","author":"H Kusumoto","year":"1988","unstructured":"Kusumoto H, Hirosawa S, Salier JP, Hagen FS, Kurachi K (1988) Human genes for complement components C1r and C1s in a close tail\u2010to\u2010tail arrangement. Proc Natl Acad Sci USA 85: 7307\u20137311","journal-title":"Proc Natl Acad Sci USA"},{"key":"BFEMMM202317405_CR37","doi-asserted-by":"crossref","first-page":"786","DOI":"10.1038\/nm.3869","volume":"21","author":"DR Lemos","year":"2015","unstructured":"Lemos DR, Babaeijandaghi F, Low M, Chang C\u2010K, Lee ST, Fiore D, Zhang R\u2010H, Natarajan A, Nedospasov SA, Rossi FMV (2015) Nilotinib reduces muscle fibrosis in chronic muscle injury by promoting TNF\u2010mediated apoptosis of fibro\/adipogenic progenitors. Nat Med 21: 786\u2013794","journal-title":"Nat Med"},{"key":"BFEMMM202317405_CR38","doi-asserted-by":"crossref","first-page":"1612","DOI":"10.1038\/nprot.2015.110","volume":"10","author":"L Liu","year":"2015","unstructured":"Liu L, Cheung TH, Charville GW, Rando TA (2015) Isolation of skeletal muscle stem cells by fluorescence\u2010activated cell sorting. Nat Protoc 10: 1612\u20131624","journal-title":"Nat Protoc"},{"key":"BFEMMM202317405_CR39","doi-asserted-by":"crossref","first-page":"351","DOI":"10.3988\/jcn.2016.12.3.351","volume":"12","author":"F Liu","year":"2016","unstructured":"Liu F, Liang Z, Xu J, Li W, Zhao D, Zhao Y, Yan C (2016) Activation of the wnt\/\u03b2\u2010catenin signaling pathway in polymyositis, dermatomyositis and Duchenne muscular dystrophy. J Clin Neurol 12: 351\u2013360","journal-title":"J Clin Neurol"},{"key":"BFEMMM202317405_CR40","doi-asserted-by":"crossref","first-page":"228","DOI":"10.1002\/cbin.10705","volume":"41","author":"X Liu","year":"2017","unstructured":"Liu X, Liu Y, Zhao L, Zeng Z, Xiao W, Chen P (2017) Macrophage depletion impairs skeletal muscle regeneration: the roles of regulatory factors for muscle regeneration. Cell Biol Int 41: 228\u2013238","journal-title":"Cell Biol Int"},{"key":"BFEMMM202317405_CR41","doi-asserted-by":"crossref","first-page":"451","DOI":"10.1016\/S0140-6736(17)32160-8","volume":"391","author":"CM McDonald","year":"2018","unstructured":"McDonald CM, Henricson EK, Abresch RT, Duong T, Joyce NC, Hu F, Clemens PR, Hoffman EP, Cnaan A, Gordish\u2010Dressman H et\u00a0al (2018) Long\u2010term effects of glucocorticoids on function, quality of life, and survival in patients with Duchenne muscular dystrophy: a prospective cohort study. Lancet 391: 451\u2013461","journal-title":"Lancet"},{"key":"BFEMMM202317405_CR42","doi-asserted-by":"crossref","first-page":"195","DOI":"10.1242\/dmm.018424","volume":"8","author":"JW McGreevy","year":"2015","unstructured":"McGreevy JW, Hakim CH, McIntosh MA, Duan D (2015) Animal models of Duchenne muscular dystrophy: from basic mechanisms to gene therapy. Dis Model Mech 8: 195\u2013213","journal-title":"Dis Model Mech"},{"key":"BFEMMM202317405_CR43","doi-asserted-by":"crossref","first-page":"2025","DOI":"10.1016\/S0140-6736(19)32910-1","volume":"394","author":"E Mercuri","year":"2019","unstructured":"Mercuri E, B\u00f6nnemann CG, Muntoni F (2019) Muscular dystrophies. Lancet 394: 2025\u20132038","journal-title":"Lancet"},{"key":"BFEMMM202317405_CR44","doi-asserted-by":"crossref","DOI":"10.1098\/rsob.210110","volume":"11","author":"T Molina","year":"2021","unstructured":"Molina T, Fabre P, Dumont NA (2021) Fibro\u2010adipogenic progenitors in skeletal muscle homeostasis, regeneration and diseases. Open Biol 11: 210110","journal-title":"Open Biol"},{"key":"BFEMMM202317405_CR45","doi-asserted-by":"crossref","first-page":"626","DOI":"10.1002\/emmm.201202096","volume":"5","author":"C Mozzetta","year":"2013","unstructured":"Mozzetta C, Consalvi S, Saccone V, Tierney M, Diamantini A, Mitchell KJ, Marazzi G, Borsellino G, Battistini L, Sassoon D et\u00a0al (2013) Fibroadipogenic progenitors mediate the ability of HDAC inhibitors to promote regeneration in dystrophic muscles of young, but not old Mdx mice. EMBO Mol Med 5: 626\u2013639","journal-title":"EMBO Mol Med"},{"key":"BFEMMM202317405_CR46","doi-asserted-by":"crossref","first-page":"276","DOI":"10.1038\/nature20160","volume":"540","author":"AA Mueller","year":"2016","unstructured":"Mueller AA, van Velthoven CT, Fukumoto KD, Cheung TH, Rando TA (2016) Intronic polyadenylation of PDGFR\u03b1 in resident stem cells attenuates muscle fibrosis. Nature 540: 276\u2013279","journal-title":"Nature"},{"key":"BFEMMM202317405_CR47","doi-asserted-by":"crossref","first-page":"385","DOI":"10.1016\/0885-4505(87)90052-1","volume":"37","author":"N Nagao","year":"1987","unstructured":"Nagao N, Shomura I, Sawada Y (1987) Serum activity of C1 inactivator in Duchenne\u2010type progressive muscular dystrophy. Biochem Med Metab Biol 37: 385\u2013388","journal-title":"Biochem Med Metab Biol"},{"key":"BFEMMM202317405_CR48","doi-asserted-by":"crossref","first-page":"1298","DOI":"10.1016\/j.cell.2012.03.047","volume":"149","author":"AT Naito","year":"2012","unstructured":"Naito AT, Sumida T, Nomura S, Liu M\u2010LL, Higo T, Nakagawa A, Okada K, Sakai T, Hashimoto A, Hara Y et\u00a0al (2012) Complement C1q activates canonical Wnt signaling and promotes aging\u2010related phenotypes. Cell 149: 1298\u20131313","journal-title":"Cell"},{"key":"BFEMMM202317405_CR49","doi-asserted-by":"crossref","first-page":"75","DOI":"10.1006\/mgme.2001.3220","volume":"74","author":"KF O'Brien","year":"2001","unstructured":"O'Brien KF, Kunkel LM (2001) Dystrophin and muscular dystrophy: past, present, and future. Mol Genet Metab 74: 75\u201388","journal-title":"Mol Genet Metab"},{"key":"BFEMMM202317405_CR50","doi-asserted-by":"crossref","first-page":"1046","DOI":"10.1016\/j.stemcr.2015.04.007","volume":"4","author":"P Pessina","year":"2015","unstructured":"Pessina P, Kharraz Y, Jard\u00ed M, Fukada S, Serrano AL, Perdiguero E, Mu\u00f1oz\u2010C\u00e1noves P (2015) Fibrogenic cell plasticity blunts tissue regeneration and aggravates muscular dystrophy. Stem Cell Reports 4: 1046\u20131060","journal-title":"Stem Cell Reports"},{"key":"BFEMMM202317405_CR51","doi-asserted-by":"crossref","first-page":"263","DOI":"10.1093\/hmg\/11.3.263","volume":"11","author":"JD Porter","year":"2002","unstructured":"Porter JD, Khanna S, Kaminski HJ, Rao JS, Merriam AP, Richmonds CR, Leahy P, Li J, Guo W, Andrade FH (2002) A chronic inflammatory response dominates the skeletal muscle molecular signature in dystrophin\u2010deficient mdx mice. Hum Mol Genet 11: 263\u2013272","journal-title":"Hum Mol Genet"},{"key":"BFEMMM202317405_CR52","volume":"17","author":"DU Purcu","year":"2022","unstructured":"Purcu DU, Korkmaz A, Gunalp S, Helvaci DG, Erdal Y, Dogan Y, Suner A, Wingender G, Sag D (2022) Effect of stimulation time on the expression of human macrophage polarization markers. PLoS ONE 17: e0265196","journal-title":"PLoS ONE"},{"key":"BFEMMM202317405_CR53","doi-asserted-by":"crossref","first-page":"1275","DOI":"10.1083\/jcb.125.6.1275","volume":"125","author":"TA Rando","year":"1994","unstructured":"Rando TA, Blau HM (1994) Primary mouse myoblast purification, characterization, and transplantation for cell\u2010mediated gene therapy. J Cell Biol 125: 1275\u20131287","journal-title":"J Cell Biol"},{"key":"BFEMMM202317405_CR54","doi-asserted-by":"crossref","first-page":"225","DOI":"10.1016\/j.clim.2015.08.009","volume":"161","author":"ES Reis","year":"2015","unstructured":"Reis ES, Mastellos DC, Yancopoulou D, Risitano AM, Ricklin D, Lambris JD (2015) Applying complement therapeutics to rare diseases. Clin Immunol 161: 225\u2013240","journal-title":"Clin Immunol"},{"key":"BFEMMM202317405_CR55","doi-asserted-by":"crossref","DOI":"10.1186\/2044-5040-1-28","volume":"1","author":"J Scharner","year":"2011","unstructured":"Scharner J, Zammit PS (2011) The muscle satellite cell at 50: the formative years. Skelet Muscle 1: 28","journal-title":"Skelet Muscle"},{"key":"BFEMMM202317405_CR56","doi-asserted-by":"crossref","first-page":"481","DOI":"10.1042\/bj2740481","volume":"274","author":"GC Sellar","year":"1991","unstructured":"Sellar GC, Blake DJ, Reid KBM (1991) Characterization and organization of the genes encoding the A\u2010, B\u2010 and C\u2010chains of human complement subcomponent C1q. The complete derived amino acid sequence of human C1q. Biochem J 274: 481\u2013490","journal-title":"Biochem J"},{"key":"BFEMMM202317405_CR57","first-page":"1107","volume":"266","author":"HH Seo","year":"2018","unstructured":"Seo HH, Lee S, Lee CY, Lee J, Shin S, Song BW, Kim IK, Choi JW, Lim S, Kim SW et\u00a0al (2018) Multipoint targeting of TGF\u2010\u03b2\/Wnt transactivation circuit with microRNA 384\u20105p for cardiac fibrosis. Cell Death Differ 266: 1107\u20131123","journal-title":"Cell Death Differ"},{"key":"BFEMMM202317405_CR58","doi-asserted-by":"crossref","first-page":"3050","DOI":"10.1016\/j.yexcr.2010.05.035","volume":"316","author":"AL Serrano","year":"2010","unstructured":"Serrano AL, Munoz\u2010Canoves P (2010) Regulation and dysregulation of fibrosis in skeletal muscle. Exp Cell Res 316: 3050\u20133058","journal-title":"Exp Cell Res"},{"key":"BFEMMM202317405_CR59","doi-asserted-by":"crossref","first-page":"141","DOI":"10.1016\/0022-510X(87)90091-8","volume":"81","author":"CA Sewry","year":"1987","unstructured":"Sewry CA, Dubowitz V, Abraha A, Luzio JP, Campbell AK (1987) Immunocytochemical localisation of complement components C8 and C9 in human diseased muscle. The role of complement in muscle fibre damage. J Neurol Sci 81: 141\u2013153","journal-title":"J Neurol Sci"},{"key":"BFEMMM202317405_CR60","doi-asserted-by":"crossref","first-page":"405","DOI":"10.1002\/jcp.21212","volume":"214","author":"W Shen","year":"2008","unstructured":"Shen W, Li Y, Zhu J, Schwendener R, Huard J (2008) Interaction between macrophages, TGF\u2010beta1, and the COX\u20102 pathway during the inflammatory phase of skeletal muscle healing after injury. J Cell Physiol 214: 405\u2013412","journal-title":"J Cell Physiol"},{"key":"BFEMMM202317405_CR61","doi-asserted-by":"crossref","first-page":"204","DOI":"10.1038\/s41580-021-00421-2","volume":"23","author":"P Sousa\u2010Victor","year":"2022","unstructured":"Sousa\u2010Victor P, Garc\u00eda\u2010Prat L, Mu\u00f1oz\u2010C\u00e1noves P (2022) Control of satellite cell function in muscle regeneration and its disruption in ageing. Nat Rev Mol Cell Biol 23: 204\u2013226","journal-title":"Nat Rev Mol Cell Biol"},{"key":"BFEMMM202317405_CR62","doi-asserted-by":"crossref","first-page":"41","DOI":"10.1212\/WNL.50.1.41","volume":"50","author":"S Spuler","year":"1998","unstructured":"Spuler S, Engel AG (1998) Unexpected sarcolemmal complement membrane attack complex deposits on nonnecrotic muscle fibers in muscular dystrophies. Neurology 50: 41\u201346","journal-title":"Neurology"},{"key":"BFEMMM202317405_CR63","doi-asserted-by":"crossref","first-page":"6241","DOI":"10.1038\/ncomms7241","volume":"6","author":"T Sumida","year":"2015","unstructured":"Sumida T, Naito AT, Nomura S, Nakagawa A, Higo T, Hashimoto A, Okada K, Sakai T, Ito M, Yamaguchi T et\u00a0al (2015) Complement C1q\u2010induced activation of \u03b2\u2010catenin signalling causes hypertensive arterial remodelling. Nat Commun 6: 6241","journal-title":"Nat Commun"},{"key":"BFEMMM202317405_CR64","doi-asserted-by":"crossref","first-page":"367","DOI":"10.1038\/s41586-018-0590-4","volume":"562","author":"Tabula Muris Consortium, Overall coordination, Logistical coordination, Organ collection and processing, Library preparation and sequencing, Computational data analysis, Cell type annotation, Writing group, Supplemental text writing group & Principal investigators","year":"2018","unstructured":"Tabula Muris Consortium, Overall coordination, Logistical coordination, Organ collection and processing, Library preparation and sequencing, Computational data analysis, Cell type annotation, Writing group, Supplemental text writing group & Principal investigators (2018) Single\u2010cell transcriptomics of 20 mouse organs creates a Tabula Muris. Nature 562: 367\u2013372","journal-title":"Nature"},{"key":"BFEMMM202317405_CR65","doi-asserted-by":"crossref","first-page":"30","DOI":"10.1016\/j.metabol.2015.10.013","volume":"65","author":"H Tao","year":"2016","unstructured":"Tao H, Yang J\u2010J, Shi K\u2010H, Li J (2016) Wnt signaling pathway in cardiac fibrosis: new insights and directions. Metabolism 65: 30\u201340","journal-title":"Metabolism"},{"key":"BFEMMM202317405_CR66","doi-asserted-by":"crossref","first-page":"R1173","DOI":"10.1152\/ajpregu.00735.2009","volume":"298","author":"JG Tidball","year":"2010","unstructured":"Tidball JG, Villalta SA (2010) Regulatory interactions between muscle and the immune system during muscle regeneration. Am J Physiol Regul Integr Comp Physiol 298: R1173\u2013R1187","journal-title":"Am J Physiol Regul Integr Comp Physiol"},{"key":"BFEMMM202317405_CR67","doi-asserted-by":"crossref","first-page":"C939","DOI":"10.1152\/ajpcell.00253.2010","volume":"299","author":"F Trensz","year":"2010","unstructured":"Trensz F, Haroun S, Cloutier A, Richter MV, Grenier G (2010) A muscle resident cell population promotes fibrosis in hindlimb skeletal muscles of mdx mice through the Wnt canonical pathway. Am J Physiol Cell Physiol 299: C939\u2013C947","journal-title":"Am J Physiol Cell Physiol"},{"key":"BFEMMM202317405_CR68","volume":"9","author":"L Tripodi","year":"2021","unstructured":"Tripodi L, Villa C, Molinaro D, Torrente Y, Farini A (2021) The immune system in Duchenne muscular dystrophy pathogenesis. Biomedicine 9: 1447","journal-title":"Biomedicine"},{"key":"BFEMMM202317405_CR69","doi-asserted-by":"crossref","first-page":"143","DOI":"10.1038\/ncb2014","volume":"12","author":"A Uezumi","year":"2010","unstructured":"Uezumi A, Fukada S, Yamamoto N, Takeda S, Tsuchida K (2010) Mesenchymal progenitors distinct from satellite cells contribute to ectopic fat cell formation in skeletal muscle. Nat Cell Biol 12: 143\u2013152","journal-title":"Nat Cell Biol"},{"key":"BFEMMM202317405_CR70","doi-asserted-by":"crossref","first-page":"3654","DOI":"10.1242\/jcs.086629","volume":"124","author":"A Uezumi","year":"2011","unstructured":"Uezumi A, Ito T, Morikawa D, Shimizu N, Yoneda T, Segawa M, Yamaguchi M, Ogawa R, Matev MM, Miyagoe\u2010Suzuki Y et\u00a0al (2011) Fibrosis and adipogenesis originate from a common mesenchymal progenitor in skeletal muscle. J Cell Sci 124: 3654\u20133664","journal-title":"J Cell Sci"},{"key":"BFEMMM202317405_CR71","doi-asserted-by":"crossref","first-page":"482","DOI":"10.1093\/hmg\/ddn376","volume":"18","author":"SA Villalta","year":"2009","unstructured":"Villalta SA, Nguyen HX, Deng B, Gotoh T, Tidbal JG (2009) Shifts in macrophage phenotypes and macrophage competition for arginine metabolism affect the severity of muscle pathology in muscular dystrophy. Hum Mol Genet 18: 482\u2013496","journal-title":"Hum Mol Genet"},{"key":"BFEMMM202317405_CR72","volume":"10","author":"X Wang","year":"2022","unstructured":"Wang X, Zhou L (2022) The many roles of macrophages in skeletal muscle injury and repair. Front Cell Dev Biol 10: 952249","journal-title":"Front Cell Dev Biol"},{"key":"BFEMMM202317405_CR73","doi-asserted-by":"crossref","first-page":"4750","DOI":"10.4049\/jimmunol.1601308","volume":"197","author":"X Wang","year":"2016","unstructured":"Wang X, Zhao W, Ransohoff RM, Zhou L (2016) Identification and function of fibrocytes in skeletal muscle injury repair and muscular dystrophy. J Immunol 197: 4750\u20134761","journal-title":"J Immunol"},{"key":"BFEMMM202317405_CR74","doi-asserted-by":"crossref","first-page":"20729","DOI":"10.1073\/pnas.1915950117","volume":"117","author":"X Wang","year":"2020","unstructured":"Wang X, Sathe AA, Smith GR, Ruf\u2010Zamojski F, Nair V, Lavine KJ, Xing C, Sealfon SC, Zhou L (2020) Heterogeneous origins and functions of mouse skeletal muscle\u2010resident macrophages. Proc Natl Acad Sci USA 117: 20729\u201320740","journal-title":"Proc Natl Acad Sci USA"},{"key":"BFEMMM202317405_CR75","doi-asserted-by":"crossref","first-page":"135","DOI":"10.1016\/j.devcel.2018.06.018","volume":"46","author":"MN Wosczyna","year":"2018","unstructured":"Wosczyna MN, Rando TA (2018) A muscle stem cell support group: coordinated cellular responses in muscle regeneration. Dev Cell 46: 135\u2013143","journal-title":"Dev Cell"},{"key":"BFEMMM202317405_CR76","doi-asserted-by":"crossref","first-page":"2029","DOI":"10.1016\/j.celrep.2019.04.074","volume":"27","author":"MN Wosczyna","year":"2019","unstructured":"Wosczyna MN, Konishi CT, Perez Carbajal EE, Wang TT, Walsh RA, Gan Q, Wagner MW, Rando TA (2019) Mesenchymal stromal cells are required for regeneration and homeostatic maintenance of skeletal muscle. Cell Rep 27: 2029\u20132035","journal-title":"Cell Rep"}],"container-title":["EMBO Molecular Medicine"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.15252\/emmm.202317405.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/link.springer.com\/article\/10.15252\/emmm.202317405\/fulltext.html","content-type":"text\/html","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/www.embopress.org\/doi\/pdf\/10.15252\/emmm.202317405","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"},{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.15252\/emmm.202317405.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,5,18]],"date-time":"2026-05-18T06:47:49Z","timestamp":1779086869000},"score":1,"resource":{"primary":{"URL":"https:\/\/link.springer.com\/10.15252\/emmm.202317405"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2023,11,6]]},"references-count":76,"journal-issue":{"issue":"12","published-print":{"date-parts":[[2023,12,7]]}},"alternative-id":["BFEMMM202317405"],"URL":"https:\/\/doi.org\/10.15252\/emmm.202317405","relation":{},"ISSN":["1757-4676","1757-4684"],"issn-type":[{"value":"1757-4676","type":"print"},{"value":"1757-4684","type":"electronic"}],"subject":[],"published":{"date-parts":[[2023,11,6]]},"assertion":[{"value":"10 January 2023","order":1,"name":"received","label":"Received","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"10 October 2023","order":2,"name":"revised","label":"Revised","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"11 October 2023","order":3,"name":"accepted","label":"Accepted","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"6 November 2023","order":4,"name":"first_online","label":"First Online","group":{"name":"ArticleHistory","label":"Article History"}}],"article-number":"EMMM202317405"}}