1. Treatment with soluble activin type IIB-receptor improves bone mass and strength in a mouse model of Duchenne muscular dystrophy
- Author
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Tero, Puolakkainen, Hongqian, Ma, Heikki, Kainulainen, Arja, Pasternack, Timo, Rantalainen, Olli, Ritvos, Kristiina, Heikinheimo, Juha J, Hulmi, and Riku, Kiviranta
- Subjects
Male ,Activin Receptors, Type II ,Drug Evaluation, Preclinical ,Osteoclasts ,Bone μCT ,Bone and Bones ,Mice ,TGF-βs ,Bone Density ,Physical Conditioning, Animal ,Animals ,Bone Resorption ,Muscle, Skeletal ,Exercise ,Osteoblasts ,Organ Size ,Muscular Dystrophy, Animal ,Combined Modality Therapy ,Bone-muscle interactions ,Animal models ,Mice, Inbred C57BL ,Muscular Dystrophy, Duchenne ,Disease Models, Animal ,Solubility ,Mice, Inbred mdx ,Research Article - Abstract
Background Inhibition of activin/myostatin pathway has emerged as a novel approach to increase muscle mass and bone strength. Duchenne muscular dystrophy (DMD) is a neuromuscular disorder that leads to progressive muscle degeneration and also high incidence of fractures. The aim of our study was to test whether inhibition of activin receptor IIB ligands with or without exercise could improve bone strength in the mdx mouse model for DMD. Methods Thirty-two mdx mice were divided to running and non-running groups and to receive either PBS control or soluble activin type IIB-receptor (ActRIIB-Fc) once weekly for 7 weeks. Results Treatment of mdx mice with ActRIIB-Fc resulted in significantly increased body and muscle weights in both sedentary and exercising mice. Femoral μCT analysis showed increased bone volume and trabecular number (BV/TV +80%, Tb.N +70%, P
- Published
- 2016