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Cited by 427

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Differentiation of pluripotent stem cells to muscle fiber to model Duchenne muscular dystrophy

Open access
Chal, Jérome, Oginuma, Masayuki, Al Tanoury, Ziad, Gobert, Bénédicte, Sumara, Olga, Hick, Aurore, Bousson, Fanny, Zidouni, Yasmine, Mursch, Caroline, Moncuquet, Philippe, Tassy, Olivier, Vincent, Stéphane, Miyanari, Ayako, Bera, Agata, Garnier, Jean-Marie, Guevara, Getzabel, Hestin, Marie, Kennedy, Leif, Hayashi, Shinichiro, Drayton, Bernadette, Cherrier, Thomas, Gayraud-Morel, Barbara, Gussoni, Emanuela, Relaix, Frédéric, Tajbakhsh, Shahragim, Pourquié, Olivier·2015·Nature Biotechnology

Abstract

During embryonic development, skeletal muscles arise from somites, which derive from the presomitic mesoderm (PSM). Using PSM development as a guide, we establish conditions for the differentiation of monolayer cultures of mouse embryonic stem (ES) cells into PSM-like cells without the introduction of transgenes or cell sorting. We show that primary and secondary skeletal myogenesis can be recapitulated in vitro from the PSM-like cells, providing an efficient, serum-free protocol for the generation of striated, contractile fibers from mouse and human pluripotent cells. The mouse ES cells also differentiate into Pax7+ cells with satellite cell characteristics, including the ability to form dystrophin+ fibers when grafted into muscles of dystrophin-deficient mdx mice, a model of Duchenne muscular dystrophy (DMD). Fibers derived from ES cells of mdx mice exhibit an abnormal branched phenotype resembling that described in vivo, thus providing an attractive model to study the origin of the pathological defects associated with DMD.

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Metadata

Authors
Chal, J., Oginuma, M., Al Tanoury, Z., Gobert, B., Sumara, O., Hick, A., Bousson, F., Zidouni, Y., Mursch, C., Moncuquet, P., Tassy, O., Vincent, S., Miyanari, A., Bera, A., Garnier, J.-M., Guevara, G., Hestin, M., Kennedy, L., Hayashi, S., Drayton, B., Cherrier, T., Gayraud-Morel, B., Gussoni, E., Relaix, F., Tajbakhsh, S., & Pourquié, O.
Venue
Nature Biotechnology
Published
2015-09
Volume
33
Issue
9
Pages
962-969
ISSN
  • 0733-222X (ISSN-L)
  • 1087-0156
  • 1546-1696
OpenAlex
W1953958400
Cited by
427
References
65
FWCI
14.02
Type
journal article
Language
en
Open access
green

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CoChat. (2026). Citation check: Differentiation of pluripotent stem cells to muscle fiber to model Duchenne muscular dystrophy. https://app.cochat.ai/papers/10.1038/nbt.3297
The paper
Chal, J., Oginuma, M., Al Tanoury, Z., Gobert, B., Sumara, O., Hick, A., Bousson, F., Zidouni, Y., Mursch, C., Moncuquet, P., Tassy, O., Vincent, S., Miyanari, A., Bera, A., Garnier, J.-M., Guevara, G., Hestin, M., Kennedy, L., Hayashi, S., . . . Pourquié, O. (2015). Differentiation of pluripotent stem cells to muscle fiber to model Duchenne muscular dystrophy. Nature Biotechnology. https://doi.org/10.1038/nbt.3297

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Contains information from the Semantic Scholar Academic Graph Datasets, made available under the ODC Attribution License (ODC-BY 1.0).

Semantic Scholar’s data is described in: Kinney, R., et al. (2023). The Semantic Scholar Open Data Platform. arXiv:2301.10140.