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

Douglas DiGirolamo, Vandana Singhal, Xiaoli Chang, Se Jin Lee, Emily L. Germain-Lee

Research output: Contribution to journalArticle

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 III OI (oim). Treatment of 12-week-old oim mice with ACVR2B for 4 weeks resulted in significant increases in both bone and muscle that were similar to those observed in healthy, wild-type littermates. This proof of concept study provides encouraging evidence for a holistic approach to treating the deleterious consequences of OI in the musculoskeletal system.

Original languageEnglish (US)
Article number14042
JournalBone Research
Volume3
DOIs
StatePublished - 2015

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Activin Receptors
Osteogenesis Imperfecta
Skeletal Muscle
Bone and Bones
Muscles
Musculoskeletal System
Bone Fractures
Muscle Weakness
Connective Tissue
Fatigue
Exercise
Pathology
Therapeutics

ASJC Scopus subject areas

  • Endocrinology, Diabetes and Metabolism
  • Histology
  • Physiology

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Administration of soluble activin receptor 2B increases bone and muscle mass in a mouse model of osteogenesis imperfecta. / DiGirolamo, Douglas; Singhal, Vandana; Chang, Xiaoli; Lee, Se Jin; Germain-Lee, Emily L.

In: Bone Research, Vol. 3, 14042, 2015.

Research output: Contribution to journalArticle

DiGirolamo, Douglas ; Singhal, Vandana ; Chang, Xiaoli ; Lee, Se Jin ; Germain-Lee, Emily L. / Administration of soluble activin receptor 2B increases bone and muscle mass in a mouse model of osteogenesis imperfecta. In: Bone Research. 2015 ; Vol. 3.
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