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In this study we investigated the effects of soluble activin type IIB receptor (sActRIIB\u2010mFc) on muscle mass and function in 2 distinct mouse models of OI: osteogenesis imperfecta murine (<jats:italic>oim<\/jats:italic>) and <jats:italic>+\/G610C<\/jats:italic>. <jats:italic>Methods:<\/jats:italic> Wild\u2010type (WT), <jats:italic>+\/G610C<\/jats:italic>, and <jats:italic>oim\/oim<\/jats:italic> mice were treated from 2 to 4 months of age with Tris\u2010buffered saline (vehicle) or sActRIIB\u2010mFc and their hindlimb muscles evaluated for mass, morphology, and contractile function. <jats:italic>Results:<\/jats:italic> sActRIIB\u2010mFc\u2010treated WT, <jats:italic>+\/G610C<\/jats:italic>, and <jats:italic>oim\/oim<\/jats:italic> mice had increased hindlimb muscle weights and myofiber cross\u2010sectional area compared with vehicle\u2010treated counterparts. sActRIIB\u2010mFc\u2010treated <jats:italic>oim\/oim<\/jats:italic> mice also exhibited increased contractile function relative to vehicle\u2010treated counterparts. <jats:italic>Discussion:<\/jats:italic> Blocking endogenous ActRIIB was effective at increasing muscle size in mouse models of OI, and increasing contractile function in <jats:italic>oim\/oim<\/jats:italic> mice. ActRIIB inhibitors may provide a potential mutation\u2010specific therapeutic option for compromised muscle function in OI. <jats:italic>Muscle Nerve<\/jats:italic> <jats:bold>57<\/jats:bold>: 294\u2013304, 2018<\/jats:p>","DOI":"10.1002\/mus.25706","type":"journal-article","created":{"date-parts":[[2017,5,27]],"date-time":"2017-05-27T01:06:42Z","timestamp":1495847202000},"page":"294-304","source":"Crossref","is-referenced-by-count":22,"title":["Soluble activin receptor type IIB decoy receptor differentially impacts murine osteogenesis imperfecta muscle function"],"prefix":"10.1002","volume":"57","author":[{"given":"Youngjae","family":"Jeong","sequence":"first","affiliation":[{"name":"Department of Biochemistry University of Missouri 117 Schweitzer Hall, Columbia Missouri 65211 USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Salah A.","family":"Daghlas","sequence":"additional","affiliation":[{"name":"Department of Biochemistry University of Missouri 117 Schweitzer Hall, Columbia Missouri 65211 USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Alp S.","family":"Kahveci","sequence":"additional","affiliation":[{"name":"Department of Biochemistry University of Missouri 117 Schweitzer Hall, Columbia Missouri 65211 USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Daniel","family":"Salamango","sequence":"additional","affiliation":[{"name":"Department of Biochemistry University of Missouri 117 Schweitzer Hall, Columbia Missouri 65211 USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Bettina A.","family":"Gentry","sequence":"additional","affiliation":[{"name":"Department of Veterinary Pathology University of Missouri Columbia Missouri USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Marybeth","family":"Brown","sequence":"additional","affiliation":[{"name":"Biomedical Science and Physical Therapy Program University of Missouri Columbia Missouri USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"R. 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