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2013-11-28 - Colloque/Présentation - poster - Anglais - 1 page(s)

Lancelot Céline , Carnac G, Nonclercq Denis , Delrée Paul, Laoudj-Chenivesse Dalila, Belayew Alexandra , Coppée Frédérique , "Development of fibrosis and its impact on muscle regeneration in FSHD muscle" in 11ème Journées de la Société Française de Myologie, 11, 40, Montpellier, France, 2013

  • Codes CREF : Pathologies particulières (DI3370)
  • Unités de recherche UMONS : Biologie moléculaire (M122)
  • Instituts UMONS : Institut des Sciences et Technologies de la Santé (Santé)
  • Centres UMONS : Mind & Health (CREMH)
Texte intégral :

Abstract(s) :

(Anglais) Facioscapulohumeral dystrophy (FSHD) is an inherited disorder characterized by atrophy and muscle weakness progressing in an asymmetric antero-posterior gradient. Previous publications and our histological analyses showed that affected FSHD muscles present an extensive fibrosis and the gradual replacing of muscle fibers by adipose tissue. These areas coexist with muscle fibers without major histological alterations. Despite the significant muscle damage, low muscle regeneration has been demonstrated in this disease compared to other muscular dystrophies. The extracellular matrix (ECM) is a major component of the satellite cell (SC) niche. Other muscle progenitor cells can also be recruited into this niche after muscle damage. After muscle injury, quiescent SC or recruited mesenchymal cells are activated and divide to provide myoblasts that repair the damaged muscle fibers. However changes in the ECM composition can alter muscle regeneration. We hypothesized that modifications in the ECM composition of FSHD muscles are contributing to the observed low muscle regeneration. Our preliminary results confirm an early thickening of ECM unfavorable to muscle regeneration in areas without histological abnormalities of affected FSHD muscles. As fibroblasts produce most components of the ECM, alterations of resident fibroblasts in FSHD muscles could contribute to the inefficiency of muscle regeneration. Indeed, our preliminary data suggest in vitro and in vivo deregulations of some ECM components. In order to characterize components of the ECM in FSHD muscles we established fibroblast and myoblast primary cultures from control and FSHD muscle biopsies. We isolated primary cells from muscle biopsy-derived cells by magnetic sorting with 50-nm superparamagnetic particles that are conjugated to highly specific antibody against a particular antigen on the fibroblast or myoblast surface. The cell suspension labeled with magnetic particles is applied to a column which contains a matrix composed of ferromagnetic spheres. When placed on a magnetic field, the magnetically labeled cells are retained within the column while unlabeled cells flow through. After a washing step, labeled cells are eluted from the column by removing the magnetic field. The identity of our cultures was confirmed using different cell markers (desmin, vimentin, collagen I and CD90). This material will allow us to determine whether fibrosis is an early event in FSHD and could contribute to the incorrect response of progenitor muscle cells. We will also investigate if the deregulations of the ECM in FSHD could affect muscle regeneration. Finally, we will evaluate if the causative gene(s) of FSHD could be involved in early fibrosis.