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In this study, the efficacy of INS\u20103001, an analogue of phytic acid, was tested for inhibition of ectopic calcification in an <jats:italic>Abcc6<\/jats:italic><jats:sup>\u2212\/\u2212<\/jats:sup> mouse model of PXE. In prevention study, <jats:italic>Abcc6<\/jats:italic><jats:sup>\u2212\/\u2212<\/jats:sup> mice, at 6\u00a0weeks of age, the time of onset of ectopic calcification, were treated with INS\u20103001 with 0.16, 0.8, 4, 20 or 100\u00a0mg\/kg\/day administered by subcutaneous implantation of osmotic pumps, as well as 4\u00a0mg\/kg\/day by subcutaneous injection thrice weekly or 14, 4 and 0.8\u00a0mg\/kg\/day once weekly subcutaneous injection. Mice were necropsied at 12\u00a0weeks of age. Histologic examination and quantitative calcium assay revealed that mice receiving 6\u00a0weeks of continuous INS\u20103001 administration via osmotic pumps showed dose\u2010dependent inhibition of muzzle skin calcification with complete response at 4\u00a0mg\/kg\/day and a minimum effective dose at 0.8\u00a0mg\/kg\/day. INS\u20103001 plasma concentrations were dose\u2010dependent and largely consistent during treatment for each dose. thrice weekly and once weekly subcutaneous injections of INS\u20103001 also prevented calcification. In established disease study, 12\u2010week\u2010old <jats:italic>Abcc6<\/jats:italic><jats:sup>\u2212\/\u2212<\/jats:sup> mice with extensive calcification were continuously administered INS\u20103001 at 4\u00a0mg\/kg\/day for a follow\u2010up of 12\u00a0weeks. INS\u20103001 treatment was found to stabilize existing calcification that had developed at start of treatment. These results suggest that INS\u20103001 may provide a promising preventive treatment strategy for PXE, a currently intractable ectopic calcification disorder.<\/jats:p>","DOI":"10.1111\/exd.14288","type":"journal-article","created":{"date-parts":[[2021,2,3]],"date-time":"2021-02-03T01:13:52Z","timestamp":1612314832000},"page":"853-858","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":6,"title":["A phytic acid analogue INS\u20103001 prevents ectopic calcification in an <i>Abcc6<\/i><sup>\u2212\/\u2212<\/sup> mouse model of pseudoxanthoma elasticum"],"prefix":"10.1111","volume":"30","author":[{"given":"Ida Joely","family":"Jacobs","sequence":"first","affiliation":[{"name":"Department of Dermatology and Cutaneous Biology PXE International Center of Excellence in Research and Clinical Care Jefferson Institute of Molecular Medicine Sidney Kimmel Medical College Thomas Jefferson University Philadelphia PA USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Diana","family":"Li","sequence":"additional","affiliation":[{"name":"Department of Dermatology and Cutaneous Biology PXE International Center of Excellence in Research and Clinical Care Jefferson Institute of Molecular Medicine Sidney Kimmel Medical College Thomas Jefferson University Philadelphia PA USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mattias E.","family":"Ivarsson","sequence":"additional","affiliation":[{"name":"Inositec Inc. 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