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 |
|---|
- Kamel Mamchaoui, Capucine Trollet, Anne Bigot, Elisa Negroni, Soraya Chaouch, et al.. Immortalized pathological human myoblasts: towards a universal tool for the study of neuromuscular disorders.. Skeletal Muscle, 2011, 1 (1), pp.34. ⟨10.1186/2044-5040-1-34⟩. ⟨inserm-00651121⟩
- Martín Baraibar, Janek Hyzewicz, Adelina Rogowska-Wrzesinska, Romain Ladouce, Peter Roepstorff, et al.. Oxidative stress-induced proteome alterations target different cellular pathways in human myoblasts. Free Radical Biology and Medicine, 2011, 51 (8), pp.1522-1532. ⟨10.1016/j.freeradbiomed.2011.06.032⟩. ⟨hal-02148122⟩
- Pierre-Francois Pradat, Aude Barani, Julia Wanschitz, Odile Dubourg, Anne Lombes, et al.. Abnormalities of satellite cells function in amyotrophic lateral sclerosis. Amyotrophic Lateral Sclerosis and Other Motor Neuron Disorders, 2011, 12 (4), pp.264-271. ⟨10.3109/17482968.2011.566618⟩. ⟨hal-00744437⟩
- Bidisha Sinha, Darius Koester, Richard Ruez, Pauline Gonnord, Michele Bastiani, et al.. Cells Respond to Mechanical Stress by Rapid Disassembly of Caveolae. Cell, 2011, 144 (3), pp.402-413. ⟨10.1016/j.cell.2010.12.031⟩. ⟨hal-00821331⟩
- Bidisha Sinha, Darius Köster, Richard Ruez, Pauline Gonnord, Michele Bastiani, et al.. Cells Respond to Mechanical Stress by Rapid Disassembly of Caveolae. Cell, 2011, 144 (3), pp.402-413. ⟨10.1016/j.cell.2010.12.031⟩. ⟨hal-02896094⟩
- Elisa Negroni, Denis Vallese, Jean-Thomas Vilquin, Gillian Butler-Browne, Vincent Mouly, et al.. Current advances in cell therapy strategies for muscular dystrophies. Expert Opinion on Biological Therapy, 2011, 11 (2), pp.157-176. ⟨10.1517/14712598.2011.542748⟩. ⟨hal-03824033⟩
- Seyed Yahya Anvar, Peter T Hoen, Andrea Venema, Barbara van Der Sluijs, Baziel van Engelen, et al.. Deregulation of the ubiquitin-proteasome system is the predominant molecular pathology in OPMD animal models and patients.. Skeletal Muscle, 2011, 1 (1), pp.15. ⟨10.1186/2044-5040-1-15⟩. ⟨inserm-00617211⟩
- Nicolas Wein, Aurélie Avril, Marc Bartoli, Cyriaque Beley, Soraya Chaouch, et al.. Efficient Bypass of Mutations in Dysferlin Deficient Patient Cells by Antisense-Induced Exon Skipping. Human Mutation, 2010, 31 (2), pp.136-142. ⟨10.1002/humu.21160⟩. ⟨hal-01610031⟩
- Elise Dargelos, Cédric Brulé, Pascal Stuelsatz, Vincent Mouly, Philippe Veschambre, et al.. Up-regulation of calcium-dependent proteolysis in human myoblasts under acute oxidative stress. Experimental Cell Research, 2010, 316 (1), pp.115-125. ⟨10.1016/j.yexcr.2009.07.025⟩. ⟨hal-02663863⟩
- Nicolas Lévy, Nicolas Wein, Florian Barthelemy, Vincent Mouly, Luis Garcia, et al.. Therapeutic exon `switching' for dysferlinopathies?. European Journal of Human Genetics, 2010, 18 (9), pp.969-970. ⟨10.1038/ejhg.2010.73⟩. ⟨hal-01610034⟩
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