Cellular and molecular orchestration in muscle regeneration, during ageing and in pathologies.
The team focuses on molecular and cellular actors involved in human in skeletal muscle regeneration, during ageing and in muscle dystrophies, particularly oculo-pharyngeal muscular dystrophy (OPMD) and Duchenne muscular dystrophy (DMD). Our approaches aim at better understandng RNA metabolism, muscle regeneration, muscle stem cells and fibrosis, in order to develop therapeutic strategies.The team has developed a solid expertise on cellular models (incuding through the MyoLine platform for immortalization taht we have set up) and on xenotransplantation using several immunodeficient mouse models and different types of grafts.
Research projects currently developed in parralel and in synergy within the team :
- Molecular mechanisms involved in OPMD and in muscle ageing
- Cell-to-cell communication during muscle regeneration, muscle ageing and fibrosis
- Development of therapeutic strategies
International associated laboratory (LIA) between France and Brazil:
Another aspect specific to our team is the development of international collaborations. In addition to numerous collaborations established over many years, we have set up an international associated laboratory between INSERM and Sorbonne University on the French side, and FIOCRUZ and the Federal University of Rio-de-Janeiro for the brazilian side. This LIA aims at synergize expertises on skeletal muscle and neuromuscular diseases from the French team and on inflammation from the Brazilian team. This LIA has been renew already once, and has produced 14 common publications, a common patent, long-term exchanges of 3 post-docs, 5 common PhD and many short term exchanges, including invited professors in both countries. A common teaching project is being set up (master and/or PhD), which will focus on innovative biotherapies for skeletal muscle.
| Name | Position | ORCID |
|---|
- Alexis Boulinguiez, Fany Roth, Hadidja Rose Mouigni, Gillian Butler-Browne, Vincent Mouly, et al.. Les agrégats nucléaires dans la dystrophie musculaire oculopharyngée. médecine/sciences, 2022, 38, pp.13 - 16. ⟨10.1051/medsci/2022175⟩. ⟨hal-03997379⟩
- Laura Muraine, Mona Bensalah, Gillian Butler-Browne, Anne Bigot, Capucine Trollet, et al.. Update on anti-fibrotic pharmacotherapies in skeletal muscle disease. Current Opinion in Pharmacology, 2022, 68, pp.102332. ⟨10.1016/j.coph.2022.102332⟩. ⟨hal-03997340⟩
- Fanny Roth, Jamila Dhiab, Alexis Boulinguiez, Hadidja-Rose Mouigni, Saskia Lassche, et al.. Assessment of PABPN1 nuclear inclusions on a large cohort of patients and in a human xenograft model of oculopharyngeal muscular dystrophy. Acta Neuropathologica, 2022, ⟨10.1007/s00401-022-02503-7⟩. ⟨hal-03832636⟩
- Alexandrine Mahoudeau, Céline Anquetil, Nozomu Tawara, Hossein Khademian, Damien Amelin, et al.. Myostatin in idiopathic inflammatory myopathies: Serum assessment and disease activity. Neuropathology and Applied Neurobiology, 2022, ⟨10.1111/nan.12849⟩. ⟨hal-03830849⟩
- Elisa Negroni, Maria Kondili, Laura Muraine, Mona Bensalah, Gillian Sandra Butler-Browne, et al.. Muscle fibro-adipogenic progenitors from a single-cell perspective: Focus on their “virtual” secretome. Frontiers in Cell and Developmental Biology, 2022, 10, ⟨10.3389/fcell.2022.952041⟩. ⟨hal-03830589⟩
- Samuel Horita, Mona Bensalah, Anne Bigot, Kamel Mamchaoui, Negroni Elisa, et al.. Laminin-111 derived peptide promotes adhesion, proliferation, differentiation and migration in human muscle cells.. Myology 2022, Sep 2022, Nice, France. ⟨hal-04010988⟩
- Mona Bensalah, Laura Muraine, Alexis Boulinguiez, Lorenzo Giordani, Victorine Albert, et al.. A negative feedback loop between fibroadipogenic progenitors and muscle fibres involving endothelin promotes human muscle fibrosis. 12th French-Japanese workshop, Sep 2022, Giverny, France. ⟨hal-04011411⟩
- Mona Bensalah, Laura Muraine, Alexis Boulinguiez, Lorenzo Giordani, Victorine Albert, et al.. Muscle fibrosis: a vicious circle between human fibroadipogenic progenitors and muscle fibers. Myology 2022, Sep 2022, Nice, France. ⟨hal-04011420⟩
- Alexandra Monceau, Dylan Moutachi, Mégane Lemaitre, Luis Garcia, Capucine Trollet, et al.. Dystrophin Restoration after Adeno-Associated Virus U7–Mediated Dmd Exon Skipping Is Modulated by Muscular Exercise in the Severe D2-Mdx Duchenne Muscular Dystrophy Murine Model. American Journal of Pathology, 2022, ⟨10.1016/j.ajpath.2022.07.016⟩. ⟨hal-03830848⟩
- Hadidja-Rose Mouigni, Nami Altin, Jamila Dhiab, Fanny Roth, Jean Lacau-St Guily, et al.. Control of PABPN1 expression in skeletal muscle. Myology 2022, Sep 2022, Nice, France. ⟨hal-04010967⟩
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