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Administration of soluble activin receptor 2B increases bone and muscle mass in a mouse model of osteogenesis imperfecta.


ABSTRACT: Osteogenesis imperfecta (OI) comprises a group of heritable connective tissue disorders generally defined by recurrent fractures, low bone mass, short stature and skeletal fragility. Beyond the skeletal complications of OI, many patients also report intolerance to physical activity, fatigue and muscle weakness. Indeed, recent studies have demonstrated that skeletal muscle is also negatively affected by OI, both directly and indirectly. Given the well-established interdependence of bone and skeletal muscle in both physiology and pathophysiology and the observations of skeletal muscle pathology in patients with OI, we investigated the therapeutic potential of simultaneous anabolic targeting of both bone and skeletal muscle using a soluble activin receptor 2B (ACVR2B) in a mouse model of type

SUBMITTER: DiGirolamo DJ 

PROVIDER: S-EPMC4472144 | biostudies-literature | 2015

REPOSITORIES: biostudies-literature