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

Producción científica: Articlerevisión exhaustiva

20 Citas (Scopus)

Resumen

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.

Idioma originalEnglish (US)
Páginas (desde-hasta)294-304
Número de páginas11
PublicaciónMuscle and Nerve
Volumen57
N.º2
DOI
EstadoPublished - feb 2018
Publicado de forma externa

ASJC Scopus subject areas

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

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