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 |
|---|
- David Suhy, Michael Graham, Capucine Trollet, Alberto Malerba, George Dickson. Reagents for treatment of oculopharyngeal muscular dystrophy (OPMD) and use thereof. France, Patent n° : 11234994. 2022. ⟨hal-04012488⟩
- Alexis Boulinguiez. Endoplasmic reticulum implication in oculopharyngeal muscular dystrophy. Club Muscle, 2022, Paris, France. ⟨hal-04011426⟩
- Capucine Trollet. Where are we now on Oculopharyngeal muscular dystrophy? -update on protein aggregation and fibrosis. Centre d'Immunologie et des Maladies Infectieuses, 2022, Paris, France. ⟨hal-04012485⟩
- Alexis Boulinguiez, Fanny Roth, Jamila Dhiab, Hadidja Rose Mouigni, Saskia Lassche, et al.. Status and role of PABPN1 nuclear aggregates in Oculopharyngeal Muscular Dystrophy. 19èmes Journées de la Société Française de Myologie, 2022, Toulouse, France. ⟨hal-04011442⟩
- Hadidja-Rose Mouigni, Nami Altin, Jamila Dhiab, Fanny Roth, Jean Lacau-St Guily, et al.. Control of PABPN1 expression in skeletal muscle. 19èmes Journées de la Société Française de Myologie, 2022, Toulouse, France. ⟨hal-04010992⟩
- Laura Muraine, Mona Bensalah, Jamila Dhiab, Stephen Gargan, Paul Dowling, et al.. Molecular and cellular actors of human muscle fibrosis among different muscular disorders. Journées Boris Ephrussi, 2022, Paris, France. ⟨hal-04011029⟩
- M Bensalah, L Muraine, A Boulinguiez, Lorenzo Giordani, V Albert, et al.. A negative feedback loop between fibroadipogenic progenitors and muscle fibres involving endothelin promotes human muscle fibrosis.. Journal of Cachexia, Sarcopenia and Muscle, In press, ⟨10.1002/jcsm.12974⟩. ⟨hal-03618341⟩
- Francesco Galli, Vincent Mouly, Gillian Butler-Browne, Giulio Cossu. Challenges in cell transplantation for muscular dystrophy. Experimental Cell Research, 2021, 409 (1), pp.112908. ⟨10.1016/j.yexcr.2021.112908⟩. ⟨hal-03832578⟩
- Hadidja-Rose Mouigni, Nami Altin, Jamila Dhiab, Fanny Roth, Jean Lacau-St Guily, et al.. Regulation of PABPN1 expression in skeletal muscle. 18èmes Journées de la Société Française de Myologie, Nov 2021, Saint-etienne, France. ⟨hal-04010908⟩
- Fanny Roth, Alexis Boulinguiez, Jamila Dhiab, Negroni Elisa, Vincent Mouly, et al.. Targeting UPR to resolve aggresome accumulation in OPMD muscle. 18èmes Journées de la Société Française de Myologie, Nov 2021, Saint Etienne, France. ⟨hal-04011367⟩
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