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Satellite cells derived from the mdx murine model of Duchenne muscular dystrophy retain their muscle regenerative capacity

Boldrin, L; Zammit, PS; Morgan, JE; (2015) Satellite cells derived from the mdx murine model of Duchenne muscular dystrophy retain their muscle regenerative capacity. Stem Cell Research , 14 (1) pp. 20-29. 10.1016/j.scr.2014.10.007. Green open access

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Abstract

Duchenne muscular dystrophy is an inherited disorder that is characterized by progressive skeletal muscle weakness and wasting, with a failure of muscle maintenance/repair mediated by satellite cells (muscle stem cells). The function of skeletal muscle stem cells resident in dystrophic muscle may be perturbed by being in an increasing pathological environment, coupled with constant demands for repairing muscle. To investigate the contribution of satellite cell exhaustion to this process, we tested the functionality of satellite cells isolated from the mdx mouse model of Duchenne muscular dystrophy. We found that satellite cells derived from young mdx mice contributed efficiently to muscle regeneration within our in vivo mouse model. To then test the effects of long-term residence in a dystrophic environment, satellite cells were isolated from aged mdx muscle. Surprisingly, they were as functional as those derived from young or aged wild type donors. Removing satellite cells from a dystrophic milieu reveals that their regenerative capacity remains both intact and similar to satellite cells derived from healthy muscle, indicating that the host environment is critical for mediating efficient satellite cell function.

Type: Article
Title: Satellite cells derived from the mdx murine model of Duchenne muscular dystrophy retain their muscle regenerative capacity
Open access status: An open access version is available from UCL Discovery
DOI: 10.1016/j.scr.2014.10.007
Publisher version: http://dx.doi.org/10.1016/j.scr.2014.10.007
Language: English
Additional information: © 2014 The Authors. Published by Elsevier B.V. This is an open access article under the CC BY license(http://creativecommons.org/licenses/by/3.0/)
UCL classification: UCL
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Population Health Sciences > UCL GOS Institute of Child Health
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Population Health Sciences > UCL GOS Institute of Child Health > Developmental Neurosciences Dept
URI: https://discovery.ucl.ac.uk/id/eprint/1455712
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