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Soluble activin receptor type IIB decoy receptor differentially impacts murine osteogenesis imperfecta muscle function.


ABSTRACT: INTRODUCTION:Osteogenesis imperfecta (OI) is characterized by skeletal fragility and muscle weakness. In this study we investigated the effects of soluble activin type IIB receptor (sActRIIB-mFc) on muscle mass and function in 2 distinct mouse models of OI: osteogenesis imperfecta murine (oim) and +/G610C. METHODS:Wild-type (WT), +/G610C, and oim/oim mice were treated from 2 to 4 months of age with Tris-buffered saline (vehicle) or sActRIIB-mFc and their hindlimb muscles evaluated for mass, morphology, and contractile function. RESULTS:sActRIIB-mFc-treated WT, +/G610C, and oim/oim mice had increased hindlimb muscle weights and myofiber cross-sectional area compared with vehicle-treated counterparts. sActRIIB-mFc-treated oim/oim mice also exhibited increased contractile function relative to vehicle-treated counterparts. DISCUSSION:Blocking endogenous ActRIIB was effective at increasing muscle size in mouse models of OI, and increasing contractile function in oim/oim mice. ActRIIB inhibitors may provide a potential mutation-specific therapeutic option for compromised muscle function in OI. Muscle Nerve 57: 294-304, 2018.

SUBMITTER: Jeong Y 

PROVIDER: S-EPMC5702601 | biostudies-literature | 2018 Feb

REPOSITORIES: biostudies-literature

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Soluble activin receptor type IIB decoy receptor differentially impacts murine osteogenesis imperfecta muscle function.

Jeong Youngjae Y   Daghlas Salah A SA   Kahveci Alp S AS   Salamango Daniel D   Gentry Bettina A BA   Brown Marybeth M   Rector R Scott RS   Pearsall R Scott RS   Phillips Charlotte L CL  

Muscle & nerve 20170615 2


<h4>Introduction</h4>Osteogenesis imperfecta (OI) is characterized by skeletal fragility and muscle weakness. In this study we investigated the effects of soluble activin type IIB receptor (sActRIIB-mFc) on muscle mass and function in 2 distinct mouse models of OI: osteogenesis imperfecta murine (oim) and +/G610C.<h4>Methods</h4>Wild-type (WT), +/G610C, and oim/oim mice were treated from 2 to 4 months of age with Tris-buffered saline (vehicle) or sActRIIB-mFc and their hindlimb muscles evaluated  ...[more]

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