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

scholarly journals Facioscapulohumeral muscular dystrophy (FSHD) region gene 1 (FRG1) is a dynamic nuclear and sarcomeric protein

2011 ◽  
Vol 81 (2) ◽  
pp. 107-118 ◽  
Author(s):  
Meredith L. Hanel ◽  
Chia-Yun Jessica Sun ◽  
Takako I. Jones ◽  
Steven W. Long ◽  
Simona Zanotti ◽  
...  
Keyword(s):  
Muscular Dystrophy ◽  
Facioscapulohumeral Muscular Dystrophy ◽  
Region Gene ◽  
Sarcomeric Protein

scholarly journals Facioscapulohumeral Muscular Dystrophy Region Gene 1 Is a Dynamic RNA-Associated and Actin-Bundling Protein

2011 ◽  
Vol 411 (2) ◽  
pp. 397-416 ◽  
Author(s):  
Chia-Yun Jessica Sun ◽  
Silvana van Koningsbruggen ◽  
Steven W. Long ◽  
Kirsten Straasheijm ◽  
Rinse Klooster ◽  
...  
Keyword(s):  
Muscular Dystrophy ◽  
Facioscapulohumeral Muscular Dystrophy ◽  
Region Gene

scholarly journals p53 convergently activates Dux/DUX4 in embryonic stem cells and in facioscapulohumeral muscular dystrophy cell models

2021 ◽  
Author(s):  
Edward J. Grow ◽  
Bradley D. Weaver ◽  
Christina M. Smith ◽  
Jingtao Guo ◽  
Paula Stein ◽  
...  
Keyword(s):  
Stem Cells ◽  
Embryonic Stem Cells ◽  
Muscular Dystrophy ◽  
Embryonic Stem ◽  
Facioscapulohumeral Muscular Dystrophy ◽  
Cell Models

Facioscapulohumeral muscular dystrophy (FSHD) region gene 1 (FRG1) expression and possible function in mouse tooth germ development

2016 ◽  
Vol 47 (4) ◽  
pp. 375-387 ◽  
Author(s):  
Kana Hasegawa ◽  
Hiroko Wada ◽  
Kengo Nagata ◽  
Hiroaki Fujiwara ◽  
Naohisa Wada ◽  
...  
Keyword(s):  
Muscular Dystrophy ◽  
Tooth Germ ◽  
Facioscapulohumeral Muscular Dystrophy ◽  
Region Gene ◽  
Tooth Germ Development

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.

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