{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,3,23]],"date-time":"2026-03-23T23:05:43Z","timestamp":1774307143854,"version":"3.50.1"},"reference-count":47,"publisher":"Wiley","issue":"8","license":[{"start":{"date-parts":[[2022,5,27]],"date-time":"2022-05-27T00:00:00Z","timestamp":1653609600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"DOI":"10.13039\/501100012190","name":"Ministry of Science and Higher Education of the Russian Federation","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100012190","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Journal Cellular Physiology"],"published-print":{"date-parts":[[2022,8]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>Facioscapulohumeral muscular dystrophy (FSHD) is a genetic disease associated with ectopic expression of the <jats:italic>DUX4<\/jats:italic> gene in skeletal muscle. Muscle degeneration in FSHD is accompanied by muscle tissue replacement with fat and connective tissue. Expression of <jats:italic>DUX4<\/jats:italic> in myoblasts stimulates mesenchymal stem cells\u00a0(MSC)\u00a0migration via the CXCR4\u2010CXCL12 axis.\u00a0MSCs\u00a0participate in adipose and connective tissue formation and can contribute to fibrosis. Here we studied the interaction between myoblasts and MSCs and the consequences of this interaction in the FSHD context. We used cell motility assays and coculture of MSCs with myoblasts to study their mutual effects on cell migration, differentiation, proliferation, and extracellular matrix formation. The growth medium conditioned by FSHD myoblasts stimulated MSCs migration 1.6\u2010fold (<jats:italic>p<\/jats:italic>\u2009&lt;\u20090.04) compared to nonconditioned medium. Blocking the CXCL12\u2010CXCR4 axis with the CXCR4 inhibitor (AMD3100) or neutralizing antibodies to CXCL12 abolished this effect. FSHD myoblasts stimulated MSC proliferation 1.5\u22122 times (<jats:italic>p<\/jats:italic>\u2009&lt;\u20090.05) compared to control myoblasts, while the presence of MSCs impaired myoblast differentiation. Under inflammatory conditions, medium conditioned by FSHD myoblasts stimulated collagen secretion by MSCs 2.2\u2010fold as compared to the nonconditioned medium, <jats:italic>p<\/jats:italic>\u2009&lt;\u20090.03. FSHD myoblasts attract MSCs via the CXCL12\u2010CXCR4 axis, stimulate MSC proliferation and collagen secretion by MSCs. Interaction between MSCs and FSHD myoblasts accounts for several important aspects of FSHD pathophysiology. The CXCL12\u2010CXCR4 axis may serve as a potential target to improve the state of the diseased muscles.<\/jats:p>","DOI":"10.1002\/jcp.30789","type":"journal-article","created":{"date-parts":[[2022,5,27]],"date-time":"2022-05-27T12:03:49Z","timestamp":1653653029000},"page":"3328-3337","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":5,"title":["Interaction between mesenchymal stem cells and myoblasts in the context of facioscapulohumeral muscular dystrophy contributes to the disease phenotype"],"prefix":"10.1002","volume":"237","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-2270-6595","authenticated-orcid":false,"given":"Ekaterina","family":"Kiseleva","sequence":"first","affiliation":[{"name":"N. K. Koltzov Institute of Developmental Biology, RAS Moscow Russia"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Olesya","family":"Serbina","sequence":"additional","affiliation":[{"name":"N. K. Koltzov Institute of Developmental Biology, RAS Moscow Russia"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-6830-149X","authenticated-orcid":false,"given":"Anna","family":"Karpukhina","sequence":"additional","affiliation":[{"name":"N. K. Koltzov Institute of Developmental Biology, RAS Moscow Russia"},{"name":"Univeristy\u00a0Paris\u2010Saclay, UMR 9018, CNRS Institut\u00a0Gustave\u2010Roussy Villejuif France"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-3809-4033","authenticated-orcid":false,"given":"Vincent","family":"Mouly","sequence":"additional","affiliation":[{"name":"Sorbonne Universit\u00e9s, UMR\u2010S 974 Center for Research in Myology Paris France"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-3101-7043","authenticated-orcid":false,"given":"Yegor S.","family":"Vassetzky","sequence":"additional","affiliation":[{"name":"N. K. 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