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

Youngjae Jeong, Salah A. Daghlas, Alp S. Kahveci, Daniel Salamango, Bettina A. Gentry, Marybeth Brown, R. Scott Rector, R. Scott Pearsall, Charlotte L. Phillips

Research output: Contribution to journalArticlepeer-review

20 Scopus citations

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.

Original languageEnglish (US)
Pages (from-to)294-304
Number of pages11
JournalMuscle and Nerve
Volume57
Issue number2
DOIs
StatePublished - Feb 2018
Externally publishedYes

Keywords

  • +/G610C
  • activin receptor type IIB
  • muscle
  • myostatin
  • osteogenesis imperfecta murine
  • peak tetanic force

ASJC Scopus subject areas

  • Physiology
  • Clinical Neurology
  • Cellular and Molecular Neuroscience
  • Physiology (medical)

Fingerprint

Dive into the research topics of 'Soluble activin receptor type IIB decoy receptor differentially impacts murine osteogenesis imperfecta muscle function'. Together they form a unique fingerprint.

Cite this