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 |
|---|
- Vered Raz, Ellen Sterrenburg, Samantha Routledge, Andrea Venema, Barbara van Der Sluijs, et al.. Nuclear entrapment and extracellular depletion of PCOLCE is associated with muscle degeneration in oculopharyngeal muscular dystrophy.. BMC Neurology, 2013, 13 (1), pp.70. ⟨10.1186/1471-2377-13-70⟩. ⟨inserm-00846336⟩
- Laura Barberi, Bianca Maria Scicchitano, Manuela de Rossi, Anne Bigot, Stephanie Duguez, et al.. Age-dependent alteration in muscle regeneration: the critical role of tissue niche.. Biogerontology, 2013, 14 (3), pp.273-92. ⟨10.1007/s10522-013-9429-4⟩. ⟨pasteur-01027544⟩
- Cyril Catelain, Stéphanie Riveron, Aurélie Papadopoulos, Nathalie Mougenot, Adeline Jacquet, et al.. Myoblasts and Embryonic Stem Cells Differentially Engraft in a Mouse Model of Genetic Dilated Cardiomyopathy. Molecular Therapy, 2013, 21 (5), pp.1064-1075. ⟨10.1038/mt.2013.15⟩. ⟨hal-03823994⟩
- Marion Desdouits, Olivier Cassar, Thierry Maisonobe, Alexandra Desrames, Achille Aouba, et al.. HTLV-1-associated inflammatory myopathies: low proviral load and moderate inflammation in 13 patients from West Indies and West Africa.. Journal of Clinical Virology, 2013, 57 (1), pp.70-6. ⟨10.1016/j.jcv.2012.12.016⟩. ⟨pasteur-01370950⟩
- Pierre Joanne, Oussama Chourbagi, Christophe Hourdé, Arnaud Ferry, Gillian Butler-Browne, et al.. Viral-mediated expression of desmin mutants to create mouse models of myofibrillar myopathy.. Skeletal Muscle, 2013, 3 (1), pp.4. ⟨10.1186/2044-5040-3-4⟩. ⟨inserm-00800537⟩
- Jing Jye Lim, Wan Zurinah Wan Ngah, Vincent Mouly, Norwahidah Abdul Karim. Reversal of Myoblast Aging by Tocotrienol Rich Fraction Posttreatment. Oxidative Medicine and Cellular Longevity, 2013, 2013, pp.978101. ⟨10.1155/2013/978101⟩. ⟨hal-01596040⟩
- Denis Vallese, Elisa Negroni, Stéphanie Duguez, Arnaud Ferry, Capucine Trollet, et al.. The Rag2⁻Il2rb⁻Dmd⁻ mouse: a novel dystrophic and immunodeficient model to assess innovating therapeutic strategies for muscular dystrophies.. Molecular Therapy, 2013, 21 (10), pp.1950-7. ⟨10.1038/mt.2013.186⟩. ⟨pasteur-01489680⟩
- Sara Gonzalez-Hilarion, Terence Beghyn, Jieshuang Jia, Nadège Debreuck, Gonzague Berte, et al.. Rescue of nonsense mutations by amlexanox in human cells.. Orphanet Journal of Rare Diseases, 2012, 7 (1), pp.58. ⟨10.1186/1750-1172-7-58⟩. ⟨inserm-00783891⟩
- Barbara Gayraud-Morel, Fabrice Chrétien, Aurélie Jory, Ramkumar Sambasivan, Elisa Negroni, et al.. Myf5 haploinsufficiency reveals distinct cell fate potentials for adult skeletal muscle stem cells.. Journal of Cell Science, 2012, 125 (Pt 7), pp.1738-49. ⟨10.1242/jcs.097006⟩. ⟨pasteur-00705770⟩
- Bouke Duijnisveld, Anne Bigot, Karel Beenakker, Débora Portilho, Vered Raz, et al.. Regenerative potential of human muscle stem cells in chronic inflammation.. Arthritis Research and Therapy, 2011, 13 (6), pp.R207. ⟨10.1186/ar3540⟩. ⟨inserm-00663198⟩
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