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Microfibrils dysfunction is responsible for the onset of various connective tissue diseases, including Marfan syndrome. Although ADAMTSL (a disintegrin and metalloproteinase with thrombospondin motifs\u2010like) 6\u03b2 is one of the fibrillin\u20101 binding proteins, the detailed mechanism underlying the involvement of ADAMTSL6\u03b2 in microfibril formation remains unclear. In this study, we created deletion mutants of ADAMTSL6\u03b2 and examined their interactions with fibrillin\u20101 assembly. Pull\u2010down assay of the ADAMTSL6\u03b2 deletion mutants and fibrillin\u20101 protein revealed that ADAMTSL6\u03b2 binds to fibrillin\u20101 through the third thrombospondin type I domain. Furthermore, we observed that formation of fibrillin\u20101 matrix assembly was enhanced in MG63 cells, expressing full\u2010length ADAMTSL6\u03b2, when compared with that of wild type MG63 cells. While MG63 cells expressing \u0394\u00a0TSP3\u2010ADAMTSL6\u03b2 form showed enhanced assembly formation, \u0394\u00a0TSP2\u2010ADAMTSL6\u03b2 form did not enhance that, indicating the difference between \u0394\u00a0TSP2\u2010\u0394\u00a0TSP3 has a critical role for fibrillin\u20101 assembly. As the difference of \u0394\u00a0TSP2\u2010\u0394\u00a0TSP3 is the third thrombospondin type I domain, we concluded that the third thrombospondin type I domain of ADAMTSL6\u03b2 influence the microfibril formation. Our data are the functional presentation of the biological role of ADAMTSL6\u03b2 in the process of microfibril formation.<\/jats:p>","DOI":"10.1002\/cbin.11337","type":"journal-article","created":{"date-parts":[[2020,3,6]],"date-time":"2020-03-06T10:28:35Z","timestamp":1583490515000},"page":"1436-1446","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":4,"title":["ADAMTSL6\u03b2 promotes fibrillin\u20101 microfibril assembly, which is possibly mediated via binding through the third thrombospondin type I domain to fibrillin\u20101"],"prefix":"10.1002","volume":"44","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-7153-9367","authenticated-orcid":false,"given":"Ai","family":"Orimoto","sequence":"first","affiliation":[{"name":"Graduate School of Science and Engineering Iwate University 4\u20103\u20105, Ueda Morioka Iwate 020\u20108551 Japan"},{"name":"Division of Operative Dentistry, Department of Restorative Dentistry Tohoku University Graduate School of Dentistry 4\u20101 Seiryo\u2010machi Sendai Miyagi 980\u20108575 Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Tomokazu","family":"Fukuda","sequence":"additional","affiliation":[{"name":"Graduate School of Science and Engineering Iwate University 4\u20103\u20105, Ueda Morioka Iwate 020\u20108551 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