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Soluble activin receptor type IIB decoy receptor differentially impacts murine osteogenesis imperfecta muscle
Youngjae Jeong1, Salah A Daghlas1, Alp S Kahveci1
1Department of Biochemistry, University of Missouri, 117 Schweitzer Hall, Columbia, Missouri, 65211, USA.
Muscle & Nerve
|May 31, 2017
Summary
Soluble activin type IIB receptor (sActRIIB-mFc) treatment increased muscle mass and function in mouse models of osteogenesis imperfecta (OI). This suggests ActRIIB inhibitors may offer a therapeutic option for OI-related muscle weakness.
Area of Science:
- Biomedical research
- Muscle physiology
- Skeletal dysplasias
Background:
- Osteogenesis imperfecta (OI) is a group of genetic disorders characterized by bone fragility and often accompanied by muscle weakness.
- Current therapeutic strategies for OI primarily focus on bone health, with limited options for addressing muscle deficits.
Purpose of the Study:
- To investigate the efficacy of soluble activin type IIB receptor (sActRIIB-mFc) in improving muscle mass and function in mouse models of OI.
- To determine if sActRIIB-mFc treatment can ameliorate muscle-related symptoms in osteogenesis imperfecta murine (oim) and +/G610C mice.
Main Methods:
- Treatment of wild-type (WT), +/G610C, and oim/oim mice with sActRIIB-mFc or vehicle from 2 to 4 months of age.
- Evaluation of hindlimb muscle mass, morphology (myofiber cross-sectional area), and contractile function in treated and control groups.
Main Results:
- sActRIIB-mFc treatment significantly increased hindlimb muscle weights and myofiber cross-sectional area in all tested mouse models of OI compared to vehicle controls.
- sActRIIB-mFc-treated oim/oim mice demonstrated enhanced hindlimb muscle contractile function relative to their vehicle-treated counterparts.
Conclusions:
- Blocking the activin type IIB receptor (ActRIIB) pathway effectively increases muscle size in mouse models of OI.
- ActRIIB inhibition presents a potential mutation-specific therapeutic approach for improving compromised muscle function in osteogenesis imperfecta.
