{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,16]],"date-time":"2026-09-16T12:03:19Z","timestamp":1789560199500,"version":"build-2803163510"},"reference-count":44,"publisher":"Oxford University Press (OUP)","issue":"4","license":[{"start":{"date-parts":[[2015,2,1]],"date-time":"2015-02-01T00:00:00Z","timestamp":1422748800000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/academic.oup.com\/pages\/standard-publication-reuse-rights"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2015,2,15]]},"abstract":"<jats:title>Abstract<\/jats:title>\n               <jats:p>Recessive dystrophic epidermolysis bullosa (RDEB) is an intractable genetic blistering skin disease in which the epithelial structure easily separates from the underlying dermis because of genetic loss of functional type VII collagen (Col7) in the cutaneous basement membrane zone. Recent studies have demonstrated that allogeneic bone marrow transplantation (BMT) ameliorates the skin blistering phenotype of RDEB patients by restoring Col7. However, the exact therapeutic mechanism of BMT in RDEB remains unclear. In this study, we investigated the roles of transplanted bone marrow\u2013derived circulating mesenchymal cells in RDEB (Col7-null) mice. In wild-type mice with prior GFP-BMT after lethal irradiation, lineage-negative\/GFP-positive (Lin\u2212\/GFP+) cells, including platelet-derived growth factor receptor \u03b1-positive (PDGFR\u03b1+) mesenchymal cells, specifically migrated to skin grafts from RDEB mice and expressed Col7. Vascular endothelial cells and follicular keratinocytes in the deep dermis of the skin grafts expressed SDF-1\u03b1, and the bone marrow\u2013derived PDGFR\u03b1+ cells expressed CXCR4 on their surface. Systemic administration of the CXCR4 antagonist AMD3100 markedly decreased the migration of bone marrow\u2013derived PDGFR\u03b1+ cells into the skin graft, resulting in persistent epidermal detachment with massive necrosis and inflammation in the skin graft of RDEB mice; without AMD3100 administration, Col7 was significantly supplemented to ameliorate the pathogenic blistering phenotype. Collectively, these data suggest that the SDF1\u03b1\/CXCR4 signaling axis induces transplanted bone marrow\u2013derived circulating PDGFR\u03b1+ mesenchymal cells to migrate and supply functional Col7 to regenerate RDEB skin.<\/jats:p>","DOI":"10.4049\/jimmunol.1400914","type":"journal-article","created":{"date-parts":[[2015,1,20]],"date-time":"2015-01-20T05:11:30Z","timestamp":1421730690000},"page":"1996-2003","source":"Crossref","is-referenced-by-count":69,"title":["Transplanted Bone Marrow\u2013Derived Circulating PDGFR\u03b1+ Cells Restore Type VII Collagen in Recessive Dystrophic Epidermolysis Bullosa Mouse Skin Graft"],"prefix":"10.1093","volume":"194","author":[{"given":"Shin","family":"Iinuma","sequence":"first","affiliation":[{"name":"Department of Stem Cell Therapy Science, Graduate School of Medicine, Osaka University , Suita 565-0871 ,","place":["Japan"]},{"name":"Division of Gene Therapy Science, Graduate School of Medicine, Osaka University , Suita 565-0871 ,","place":["Japan"]},{"name":"Department of Dermatology, Asahikawa Medical College , Asahikawa 078-8510 ,","place":["Japan"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Eriko","family":"Aikawa","sequence":"additional","affiliation":[{"name":"Department of Stem Cell Therapy Science, Graduate School of Medicine, Osaka University , Suita 565-0871 ,","place":["Japan"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Katsuto","family":"Tamai","sequence":"additional","affiliation":[{"name":"Department of Stem Cell Therapy Science, Graduate School of Medicine, Osaka University , Suita 565-0871 ,","place":["Japan"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ryo","family":"Fujita","sequence":"additional","affiliation":[{"name":"Division of Gene Therapy Science, Graduate School of Medicine, Osaka University , Suita 565-0871 ,","place":["Japan"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yasushi","family":"Kikuchi","sequence":"additional","affiliation":[{"name":"Department of Stem Cell Therapy Science, Graduate School of Medicine, Osaka University , Suita 565-0871 ,","place":["Japan"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Takenao","family":"Chino","sequence":"additional","affiliation":[{"name":"Department of Stem Cell Therapy Science, Graduate School of Medicine, Osaka University , Suita 565-0871 ,","place":["Japan"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Junichi","family":"Kikuta","sequence":"additional","affiliation":[{"name":"Immunology and Cell Biology, Graduate School of Medicine, Osaka University , Suita 565-0871 ,","place":["Japan"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"John A","family":"McGrath","sequence":"additional","affiliation":[{"name":"Department of Molecular Dermatology, King\u2019s College , London WC2R 2LS ,","place":["United Kingdom"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jouni","family":"Uitto","sequence":"additional","affiliation":[{"name":"Department of Dermatology and Cutaneous Biology, Sidney Kimmel Medical College at Thomas Jefferson University , Philadelphia, PA 19107"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Masaru","family":"Ishii","sequence":"additional","affiliation":[{"name":"Immunology and Cell Biology, Graduate School of Medicine, Osaka University , Suita 565-0871 ,","place":["Japan"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hajime","family":"Iizuka","sequence":"additional","affiliation":[{"name":"Department of Dermatology, Asahikawa Medical College , Asahikawa 078-8510 ,","place":["Japan"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yasufumi","family":"Kaneda","sequence":"additional","affiliation":[{"name":"Division of Gene Therapy Science, Graduate School of Medicine, Osaka University , Suita 565-0871 ,","place":["Japan"]}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"286","published-online":{"date-parts":[[2015,2,15]]},"reference":[{"key":"2025030705031088300_r1","doi-asserted-by":"crossref","first-page":"803","DOI":"10.2353\/ajpath.2008.070977","article-title":"Bone marrow cell transfer into fetal circulation can ameliorate genetic skin diseases by providing fibroblasts to the skin and inducing immune tolerance","volume":"173","author":"Chino","year":"2008","journal-title":"Am. 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