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 |
|---|
- Aymeric Chartier, Pierre Klein, Stéphanie Pierson, Nicolas Barbezier, Teresa Gidaro, et al.. Mitochondrial Dysfunction Reveals the Role of mRNA Poly(A) Tail Regulation in Oculopharyngeal Muscular Dystrophy Pathogenesis. PLoS Genetics, 2015, 11 (3), pp.e1005092. ⟨10.1371/journal.pgen.1005092⟩. ⟨hal-01231399⟩
- Yusuke Echigoya, Vincent Mouly, Luis Garcia, Toshifumi Yokota, William Duddy. In Silico Screening Based on Predictive Algorithms as a Design Tool for Exon Skipping Oligonucleotides in Duchenne Muscular Dystrophy. PLoS ONE, 2015, 10 (3), pp.e0120058. ⟨10.1371/journal.pone.0120058⟩. ⟨hal-01233078⟩
- Débora Portilho, Marcelo Ribeiro Alves, Gueorgui Kratassiouk, Stéphane Roche, Frédérique Magdinier, et al.. miRNA Expression in Control and FSHD Fetal Human Muscle Biopsies. PLoS ONE, 2015, 10 (2), pp.e0116853. ⟨10.1371/journal.pone.0116853⟩. ⟨hal-01219803⟩
- Marie Maurer, Sylvain Bougoin, Tali Feferman, Mélinée Frenkian, Jacky Bismuth, et al.. IL-6 and Akt are involved in muscular pathogenesis in myasthenia gravis. Acta Neuropathologica Communications, 2015, 3 (1), pp.1. ⟨10.1186/s40478-014-0179-6⟩. ⟨hal-01116534⟩
- J. Miro, A. M. Laaref, V. Rofidal, R. Lagrafeuille, S. Hem, et al.. FUBP1: a new protagonist in splicing regulation of the DMD gene. Nucleic Acids Research, 2015, 43 (4), pp.2378-2389. ⟨10.1093/nar/gkv086⟩. ⟨hal-01279092⟩
- Marine Cacheux, Ariane Blum, Muriel Sébastien, Julie Brocard, Kamel Mamchaoui, et al.. Functional characterization of a Central Core Disease RyR1 mutation (p.Y4864H) associated with quantitative defect in RyR1 protein. Journal of Neuromuscular Diseases, 2015, 202 (4), pp.421-432. ⟨10.3233/JND-150073⟩. ⟨inserm-01216714⟩
- Florian Barthelemy, Cedric Blouin, Nicolas Wein, Vincent Mouly, Sebastien Courrier, et al.. Exon 32 Skipping of Dysferlin Rescues Membrane Repair in Patients' Cells. Journal of Neuromuscular Diseases, 2015, 2 (3), pp.281-290. ⟨10.3233/JND-150109⟩. ⟨hal-01662831⟩
- Lu Zhu, Apostolos Malatras, Matthew Thorley, Idonnya Aghoghogbe, Arvind Mer, et al.. CellWhere: graphical display of interaction networks organized on subcellular localizations. Nucleic Acids Research, 2015, 43 (W1), pp.W571-W575. ⟨10.1093/nar/gkv354⟩. ⟨hal-01277971⟩
- Virginie Mariot, S. Roche, Débora Portilho, Sabrina Sacconi, Francesca Puppo, et al.. Correlation between low FAT1 expression and early affected muscle in facioscapulohumeral muscular dystrophy. Annals of Neurology, 2015, 78 (3), pp.387-400. ⟨10.1002/ana.24446⟩. ⟨hal-01431338⟩
- Inès Barthélémy, Fernanda Pinto-Mariz, Erica Yada, Loic Desquilbet, Wilson Savino, et al.. Predictive markers of clinical outcome in the GRMD dog model of Duchenne muscular dystrophy. Disease Models & Mechanisms, 2014, 7 (11), pp.1253-1261. ⟨10.1242/dmm.016014⟩. ⟨hal-01316488⟩
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