scholarly journals Skeletal muscle repair in a mouse model of nemaline myopathy

2006 ◽  
Vol 15 (17) ◽  
pp. 2603-2612 ◽  
Author(s):  
Despina Sanoudou ◽  
Mark A. Corbett ◽  
Mei Han ◽  
Majid Ghoddusi ◽  
Mai-Anh T. Nguyen ◽  
...  
Keyword(s):  
Skeletal Muscle ◽  
Mouse Model ◽  
Nemaline Myopathy ◽  
Muscle Repair

scholarly journals Functional Characterization of the Intact Diaphragm in a Nebulin-Based Nemaline Myopathy (NM) Model-Effects of the Fast Skeletal Muscle Troponin Activator tirasemtiv

2019 ◽  
Vol 20 (20) ◽  
pp. 5008
Author(s):  
Eun-Jeong Lee ◽  
Justin Kolb ◽  
Darren T. Hwee ◽  
Fady I. Malik ◽  
Henk L. Granzier
Keyword(s):  
Skeletal Muscle ◽  
Mouse Model ◽  
Isometric Force ◽  
Functional Characterization ◽  
Nemaline Myopathy ◽  
Diaphragm Muscle ◽  
Fast Skeletal Muscle ◽  
Shortening Velocity ◽  
Complex Load ◽  
Maximal Stimulation

Respiratory failure due to diaphragm dysfunction is considered a main cause of death in nemaline myopathy (NM) and we studied both isometric force and isotonic shortening of diaphragm muscle in a mouse model of nebulin-based NM (Neb cKO). A large contractile deficit was found in nebulin-deficient intact muscle that is frequency dependent, with the largest deficits at low–intermediate stimulation frequencies (e.g., a deficit of 72% at a stimulation frequency of 20 Hz). The effect of the fast skeletal muscle troponin activator (FSTA) tirasemtiv on force was examined. Tirasemtiv had a negligible effect at maximal stimulation frequencies, but greatly reduced the force deficit of the diaphragm at sub-maximal stimulation levels with an effect that was largest in Neb cKO diaphragm. As a result, the force deficit of Neb cKO diaphragm fell (from 72% to 29% at 20 Hz). Similar effects were found in in vivo experiments on the nerve-stimulated gastrocnemius muscle complex. Load-clamp experiments on diaphragm muscle showed that tirasemtiv increased the shortening velocity, and reduced the deficit in mechanical power by 33%. Thus, tirasemtiv significantly improves muscle function in a mouse model of nebulin-based nemaline myopathy.

scholarly journals Skeletal muscle repair by adult human mesenchymal stem cells from synovial membrane

2003 ◽  
Vol 160 (6) ◽  
pp. 909-918 ◽  
Author(s):  
Cosimo De Bari ◽  
Francesco Dell'Accio ◽  
Frank Vandenabeele ◽  
Joris R. Vermeesch ◽  
Jean-Marc Raymackers ◽  
...  
Keyword(s):  
Skeletal Muscle ◽  
Stem Cells ◽  
Mesenchymal Stem Cells ◽  
Mouse Model ◽  
Nude Mouse ◽  
Synovial Membrane ◽  
Myogenic Differentiation ◽  
Muscle Repair ◽  
Mouse Muscle ◽  
Adult Human

We have demonstrated previously that adult human synovial membrane-derived mesenchymal stem cells (hSM-MSCs) have myogenic potential in vitro (De Bari, C., F. Dell'Accio, P. Tylzanowski, and F.P. Luyten. 2001. Arthritis Rheum. 44:1928–1942). In the present study, we have characterized their myogenic differentiation in a nude mouse model of skeletal muscle regeneration and provide proof of principle of their potential use for muscle repair in the mdx mouse model of Duchenne muscular dystrophy. When implanted into regenerating nude mouse muscle, hSM-MSCs contributed to myofibers and to long term persisting functional satellite cells. No nuclear fusion hybrids were observed between donor human cells and host mouse muscle cells. Myogenic differentiation proceeded through a molecular cascade resembling embryonic muscle development. Differentiation was sensitive to environmental cues, since hSM-MSCs injected into the bloodstream engrafted in several tissues, but acquired the muscle phenotype only within skeletal muscle. When administered into dystrophic muscles of immunosuppressed mdx mice, hSM-MSCs restored sarcolemmal expression of dystrophin, reduced central nucleation, and rescued the expression of mouse mechano growth factor.

scholarly journals Therapeutic potential of adipose-derived stem cells and macrophages for ischemic skeletal muscle repair

2017 ◽  
Vol 12 (2) ◽  
pp. 153-167 ◽  
Author(s):  
Viktoriya Rybalko ◽  
Pei-Ling Hsieh ◽  
Laura M Ricles ◽  
Eunna Chung ◽  
Roger P Farrar ◽  
...  
Keyword(s):  
Skeletal Muscle ◽  
Stem Cells ◽  
Therapeutic Potential ◽  
Adipose Derived Stem Cells ◽  
Muscle Repair

scholarly journals Coaxing stem cells for skeletal muscle repair

2015 ◽  
Vol 84 ◽  
pp. 198-207 ◽  
Author(s):  
Karl J.A. McCullagh ◽  
Rita C.R. Perlingeiro
Keyword(s):  
Skeletal Muscle ◽  
Stem Cells ◽  
Muscle Repair

Skeletal Muscle Surrogates for the Acquisition of Muscle Repair Skills in Upper Limb Surgery

2022 ◽  
Author(s):  
leonie heskin ◽  
Rose Galvin ◽  
Jack Conroy ◽  
Oscar Traynor ◽  
Stephen Madden ◽  
...  
Keyword(s):  
Skeletal Muscle ◽  
Upper Limb ◽  
Muscle Repair ◽  
Upper Limb Surgery
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