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 |
|---|
- L. Benarroch, I. Nelson, T. Stojkovic, B Mohand Oumoussa, H. Madry, et al.. P166 Deciphering the genetic cause of oculopharyngodistal myopathy in a French cohort using Cas9-targeted long-read sequencing. 28th International Annual Congress of the World Muscle Society, Oct 2023, Charleston SC, United States. pp.S141, ⟨10.1016/j.nmd.2023.07.298⟩. ⟨hal-04280249⟩
- Barbara Tedesco, Leen Vendredy, Elias Adriaenssens, Marta Cozzi, Bob Asselbergh, et al.. HSPB8 frameshift mutant aggregates weaken chaperone-assisted selective autophagy in neuromyopathies. Autophagy, 2023, 19 (8), pp.2217-2239. ⟨10.1080/15548627.2023.2179780⟩. ⟨hal-04253799⟩
- Willem de Ridder, Geert de Vries, Kristof van Schil, Tine Deconinck, Vincent Mouly, et al.. A homozygous loss of function variant in POPDC3: From invalidating exercise intolerance to a limb-girdle muscular dystrophy phenotype. Neuromuscular Disorders, 2023, 33 (5), pp.432-439. ⟨10.1016/j.nmd.2023.04.003⟩. ⟨hal-04253823⟩
- Eline Lemerle, Jeanne Lainé, Marion Benoist, Gilles Moulay, Anne Bigot, et al.. Caveolae and Bin1 form ring-shaped platforms for T-tubule initiation. eLife, 2023, 12, ⟨10.7554/eLife.84139⟩. ⟨hal-04094370⟩
- 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, 2023, 68, pp.102332. ⟨10.1016/j.coph.2022.102332⟩. ⟨hal-04253789⟩
- 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⟩
- 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, 192 (11), pp.1604-1618. ⟨10.1016/j.ajpath.2022.07.016⟩. ⟨hal-04308024⟩
- 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⟩
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