Musculoskeletal system: development, physiology, pathologies

Principes fondamentaux de génétique, biologie moléculaire, biochimie, biologie du développement et physiologie

? Développement et croissance du système musculo-squelettique ? Pathologies génétiques du muscle : du diagnostic au traitement ? Système ostéo-articulaire ? Organisation du mouvement et de la motricité ? Métabolisme énergétique et protéique du muscle squelettique

Cours magistraux
Embryo and Foetal development of the musculoskeletal system (4h30)
The developmental origin of muscles and skeleton elements
Genetic Network controlling myogenesis
Formation of the myotube : migration/fusion/differentiation
Myofibrillogenesis: assembly, growth, and dynamics of myofibrils
Setting up the muscle types: glycolytic fast fibres versus oxidative slow fibres.
Adult myogenesis and regeneration (1h30)
Origin of satellite cells (myogenic and non-myogenic contribution)
Satellite cells niche (basal lamina)
Activation and proliferation of satellite cells during muscle growth and regeneration
Tissue cross talk governing musculoskeletal system development : (3h)
The Neuromuscular Junction : Mechanisms Regulating development and maintenance of the NMJ / impact of neuromuscular activity on fibre types and muscle metabolism.
Integrative morphogenesis and cross-tissue communication: MTJ formation, endocrine coupling of muscle and connected tissues (myokine concept)
Osteoarticular system (1h30)
Organization of motor skills (3h)
Organization of movement, nervous regulation and involvement of the cardiovascular system (1h30)
Fiber types and mechanisms of contraction (1h30)

Skeletal muscle metabolism (4h30)
Energy homeostasis and ATP Production (1h30)
Protein metabolism and synthesis (1h30)
Proteolytic systems (1h30)

Genetic muscle disorders (3h)
Etiology of genetic diseases affecting muscle development or function (in line with actors already described to be implicated in normal muscle development) (1h)
Strategies for dissecting physiopathological mechanisms (1h)
Presentation of different therapeutic approches tested in clinical trials (gene therapy, stem cell therapy, exon skipping) (1h)

Travaux Dirigés
1 TD (1h30): Publication analysis (myofibrillogenesis, sarcomerogenesis)
1 TD (1h30): Publication analysis (coordination of growth and differentiation : the limb musculoskeletal system)
1 TD (1h30) : Publication analysis on motor skills
1 TD (1h30) : Publication analysis on skeletal muscle metabolism
1 TD (1h30): Publication analysis on proof of concept of a therapeutic approach for a genetic muscle disorder
1 ED : external lecturer

Travaux pratiques
Pas de TP

? Développement et croissance du système musculo-squelettique ? Pathologies génétiques du muscle : du diagnostic au traitement ? Système ostéo-articulaire ? Organisation du mouvement et de la motricité ? Métabolisme énergétique et protéique du muscle squelettique