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Int J Mol Sci. 2021 Nov 30;22(23). doi: 10.3390/ijms222312985.

Skeletal Ryanodine Receptors Are Involved in Impaired Myogenic Differentiation in Duchenne Muscular Dystrophy Patients.

International journal of molecular sciences

Pierre Meyer, Cécile Notarnicola, Albano C Meli, Stefan Matecki, Gérald Hugon, Jérémy Salvador, Mirna Khalil, Léonard Féasson, Claude Cances, Jérôme Cottalorda, Isabelle Desguerre, Jean-Marie Cuisset, Pascal Sabouraud, Alain Lacampagne, Hugues Chevassus, François Rivier, Gilles Carnac

Affiliations

  1. PhyMedExp, University of Montpellier, Inserm, CNRS, 34295 Montpellier, France.
  2. Reference Centre for Neuromuscular Diseases AOC, Clinical Investigation Centre, Pediatric Neurology Department, Montpellier University Hospital, 34000 Montpellier, France.
  3. Clinical Investigation Center, Montpellier University Hospital, 34000 Montpellier, France.
  4. Myology Unit, Reference Center for Neuromuscular Diseases Euro-NmD, Inter-University Laboratory of Human Movement Sciences-EA7424, University Hospital of Saint-Etienne, 42055 Saint-Etienne, France.
  5. Reference Center for Neuromuscular Diseases AOC, Pediatric Neurology Department, Toulouse University Hospital, 3100 Toulouse, France.
  6. Pediatric Clinical Research Unit, Pediatric Multi-thematic Module CIC 1436, Toulouse Children's Hospital, 31300 Toulouse, France.
  7. Pediatric Orthopedic and Plastic Surgery Department, Montpellier University Hospital, 34295 Montpellier, France.
  8. Reference Center for Neuromuscular Diseases Paris Nord-Ile-de-France-Est, Pediatric Neurology Department, Necker Enfant Malades University Hospital, Assistance Publique des Hôpitaux de Paris Centre, Paris University, 75019 Paris, France.
  9. Reference Center for Neuromuscular Diseases Nord-Ile-de-France-Est, Pediatric Neurology Department, Lille University Hospital, 59000 Lille, France.
  10. Reference Center for Neuromuscular Diseases Nord-Ile-de-France-Est, Pediatric Neurology Department, Reims University Hospital, 51100 Reims, France.

PMID: 34884796 DOI: 10.3390/ijms222312985

Abstract

Duchenne muscular dystrophy (DMD) is characterized by progressive muscle wasting following repeated muscle damage and inadequate regeneration. Impaired myogenesis and differentiation play a major role in DMD as well as intracellular calcium (Ca

Keywords: Duchenne muscular dystrophy; endomysial fibrosis; human; myogenesis; ryanodine receptor

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