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Open AccessArticle

Derivation and Characterization of Immortalized Human Muscle Satellite Cell Clones from Muscular Dystrophy Patients and Healthy Individuals

1
Institut NeuroMyoGène, Université Claude Bernard Lyon 1, Université de Lyon, CNRS 5310, INSERM U1217, 69008 Lyon, France
2
Institut Cochin, INSERM U1016, CNRS UMR8104, Université Paris Descartes, Université Sorbonne Paris Cité, 75014 Paris, France
3
Laboratoire de Culture Cellulaire, Service de Génétique et Biologie Moléculaires—Hôpital Cochin, APHP.5, Assistance Publique-Hôpitaux de Paris, 75014 Paris, France
4
Sprott Center for Stem Cell Research, Ottawa Hospital Research Institute; Department of Cellular and Molecular Medicine, University of Ottawa, Ottawa, ON K1H 8M5, Canada
*
Author to whom correspondence should be addressed.
Cells 2020, 9(8), 1780; https://doi.org/10.3390/cells9081780
Received: 29 June 2020 / Revised: 17 July 2020 / Accepted: 22 July 2020 / Published: 26 July 2020
(This article belongs to the Section Stem Cells)
In Duchenne muscular dystrophy (DMD) patients, absence of dystrophin causes muscle wasting by impacting both the myofiber integrity and the properties of muscle stem cells (MuSCs). Investigation of DMD encompasses the use of MuSCs issued from human skeletal muscle. However, DMD-derived MuSC usage is restricted by the limited number of divisions that human MuSCs can undertake in vitro before losing their myogenic characteristics and by the scarcity of human material available from DMD muscle. To overcome these limitations, immortalization of MuSCs appears as a strategy. Here, we used CDK4/hTERT expression in primary MuSCs and we derived MuSC clones from a series of clinically and genetically characterized patients, including eight DMD patients with various mutations, four congenital muscular dystrophies and three age-matched control muscles. Immortalized cultures were sorted into single cells and expanded as clones into homogeneous populations. Myogenic characteristics and differentiation potential were tested for each clone. Finally, we screened various promoters to identify the preferred gene regulatory unit that should be used to ensure stable expression in the human MuSC clones. The 38 clonal immortalized myogenic cell clones provide a large collection of controls and DMD clones with various genetic defects and are available to the academic community. View Full-Text
Keywords: human muscle stem cells; immortalization; degenerative myopathies; Duchenne muscular dystrophy; congenital myopathies human muscle stem cells; immortalization; degenerative myopathies; Duchenne muscular dystrophy; congenital myopathies
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Massenet, J.; Gitiaux, C.; Magnan, M.; Cuvellier, S.; Hubas, A.; Nusbaum, P.; Dilworth, F.J.; Desguerre, I.; Chazaud, B. Derivation and Characterization of Immortalized Human Muscle Satellite Cell Clones from Muscular Dystrophy Patients and Healthy Individuals. Cells 2020, 9, 1780.

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