scholarly journals Identification of a putative pathway for the muscle homing of stem cells in a muscular dystrophy model

2003 ◽  
Vol 162 (3) ◽  
pp. 511-520 ◽  
Author(s):  
Yvan Torrente ◽  
Geoffrey Camirand ◽  
Federica Pisati ◽  
Marzia Belicchi ◽  
Barbara Rossi ◽  
...  
Keyword(s):  
Stem Cells ◽  
Muscular Dystrophy ◽  
Adhesion Molecule ◽  
Systemic Circulation ◽  
Mdx Mice ◽  
Systemic Delivery ◽  
Skeletal Myoblasts ◽  
Myogenic Stem Cells ◽  
Muscle Lesions ◽  
Cell Adhesion Molecule 1

Attempts to repair muscle damage in Duchenne muscular dystrophy (DMD) by transplanting skeletal myoblasts directly into muscles are faced with the problem of the limited migration of these cells in the muscles. The delivery of myogenic stem cells to the sites of muscle lesions via the systemic circulation is a potential alternative approach to treat this disease. Muscle-derived stem cells (MDSCs) were obtained by a MACS® multisort method. Clones of MDSCs, which were Sca-1+/CD34−/L-selectin+, were found to adhere firmly to the endothelium of mdx dystrophic muscles after i.v. or i.m. injections. The subpopulation of Sca-1+/CD34− MDSCs expressing L-selectin was called homing MDSCs (HMDSCs). Treatment of HMDSCs with antibodies against L-selectin prevented adhesion to the muscle endothelium. Importantly, we found that vascular endothelium from striate muscle of young mdx mice expresses mucosal addressin cell adhesion molecule-1 (MAdCAM-1), a ligand for L-selectin. Our results showed for the first time that the expression of the adhesion molecule L-selectin is important for muscle homing of MDSCs. This discovery will aid in the improvement of a potential therapy for muscular dystrophy based on the systemic delivery of MDSCs.

scholarly journals Human epicardium-derived cells fuse with high efficiency with skeletal myotubes and differentiate toward the skeletal muscle phenotype: a comparison study with stromal and endothelial cells

2011 ◽  
Vol 22 (5) ◽  
pp. 581-592 ◽  
Author(s):  
Antonietta Gentile ◽  
Gabriele Toietta ◽  
Vincenzo Pazzano ◽  
Vasileios D. Tsiopoulos ◽  
Ada Francesca Giglio ◽  
...  
Keyword(s):  
Endothelial Cells ◽  
High Efficiency ◽  
Low Frequency ◽  
Mdx Mice ◽  
Skeletal Myoblasts ◽  
Primary Myoblasts ◽  
Multipotent Cells ◽  
Nonmuscle Cells ◽  
Cell Adhesion Molecule 1

Recent studies have underscored a role for the epicardium as a source of multipotent cells. Here, we investigate the myogenic potential of adult human epicardium-derived cells (EPDCs) and analyze their ability to undergo skeletal myogenesis when cultured with differentiating primary myoblasts. Results are compared to those obtained with mesenchymal stromal cells (MSCs) and with endothelial cells, another mesodermal derivative. We demonstrate that EPDCs spontaneously fuse with pre-existing myotubes with an efficiency that is significantly higher than that of other cells. Although at a low frequency, endothelial cells may also contribute to myotube formation. In all cases analyzed, after entering the myotube, nonmuscle nuclei are reprogrammed to express muscle-specific genes. The fusion competence of nonmyogenic cells in vitro parallels their ability to reconstitute dystrophin expression in mdx mice. We additionally show that vascular cell adhesion molecule 1 (VCAM1) expression levels of nonmuscle cells are modulated by soluble factors secreted by skeletal myoblasts and that VCAM1 function is required for fusion to occur. Finally, treatment with interleukin (IL)-4 or IL-13, two cytokines released by differentiating myotubes, increases VCAM1 expression and enhances the rate of fusion of EPDCs and MSCs, but not that of endothelial cells.

scholarly journals Systemic Delivery of Allogenic Muscle Stem Cells Induces Long-Term Muscle Repair and Clinical Efficacy in Duchenne Muscular Dystrophy Dogs

2011 ◽  
Vol 179 (5) ◽  
pp. 2501-2518 ◽  
Author(s):  
Karl Rouger ◽  
Thibaut Larcher ◽  
Laurence Dubreil ◽  
Jack-Yves Deschamps ◽  
Caroline Le Guiner ◽  
...  
Keyword(s):  
Stem Cells ◽  
Duchenne Muscular Dystrophy ◽  
Muscular Dystrophy ◽  
Clinical Efficacy ◽  
Muscle Repair ◽  
Systemic Delivery ◽  
Muscle Stem Cells

Patient-tailored application for Duchene muscular dystrophy on mdx mice based induced mesenchymal stem cells

2014 ◽  
Vol 97 (2) ◽  
pp. 253-258 ◽  
Author(s):  
Jaemin Jeong ◽  
Kyungshin Shin ◽  
Seung Bum Lee ◽  
Dong Ryul Lee ◽  
Heechung Kwon
Keyword(s):  
Stem Cells ◽  
Mesenchymal Stem Cells ◽  
Muscular Dystrophy ◽  
Mdx Mice ◽  
Duchene Muscular Dystrophy

scholarly journals Wharton’s Jelly-Derived Mesenchymal Stem Cells Reduce Fibrosis in a Mouse Model of Duchenne Muscular Dystrophy by Upregulating microRNA 499

Biomedicines ◽  
2021 ◽  
Vol 9 (9) ◽  
pp. 1089
Author(s):  
Sang Eon Park ◽  
Jang Bin Jeong ◽  
Shin Ji Oh ◽  
Sun Jeong Kim ◽  
Hyeongseop Kim ◽  
...  
Keyword(s):  
Stem Cells ◽  
Mesenchymal Stem Cells ◽  
Duchenne Muscular Dystrophy ◽  
Muscular Dystrophy ◽  
Wharton’S Jelly ◽  
Mdx Mice ◽  
Therapeutic Effects ◽  
Western Blots ◽  
Muscle Disorders ◽  
Wharton's Jelly

The aim of this study was to evaluate the therapeutic effects and mechanisms of Wharton’s jelly-derived mesenchymal stem cells (WJ-MSCs) in an animal model of Duchenne muscular dystrophy (DMD). Mdx mice (3–5 months old) were administered five different doses of WJ-MSCs through their tail veins. A week after injection, grip strength measurements, creatine kinase (CK) assays, immunohistochemistry, and western blots were performed for comparison between healthy mice, mdx control mice, and WJ-MSC-injected mdx mice. WJ-MSCs exerted dose-dependent multisystem therapeutic effects in mdx mice, by decreasing CK, recovering normal behavior, regenerating muscle, and reducing apoptosis and fibrosis in skeletal muscle. We also confirmed that miR-499-5p is significantly downregulated in mdx mice, and that intravenous injection of WJ-MSCs enhanced its expression, leading to anti-fibrotic effects via targeting TGFβR 1 and 3. Thus, WJ-MSCs may represent novel allogeneic “off-the-shelf” cellular products for the treatment of DMD and possibly other muscle disorders.

scholarly journals Overexpression of facioscapulohumeral muscular dystrophy region gene 1 causes primary defects in myogenic stem cells

2013 ◽  
Vol 126 (10) ◽  
pp. 2236-2245 ◽  
Author(s):  
A. Xynos ◽  
M. V. Neguembor ◽  
R. Caccia ◽  
D. Licastro ◽  
A. Nonis ◽  
...  
Keyword(s):  
Stem Cells ◽  
Muscular Dystrophy ◽  
Facioscapulohumeral Muscular Dystrophy ◽  
Region Gene ◽  
Myogenic Stem Cells
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