Biomedical research institute
     
    You are here: Home / Departments / Development, Reproduction and Cancer / Team P. Maire, A. Sotiropoulos

    Neuromuscular Development, Genetics and Physiopathology

    Team leaders

     

    The adult skeletal muscle tissue is composed primarily of specialized cells, myofibers , which represent 40-50 % of body weight in humans. They have associated muscle stem cells ( CSM ) called satellite cells.

    The muscles are innervated by motoneurons , which synaptic activities lead to muscle contractions and motor activity . In Europe, more than 15 million people are affected by muscle diseases, that always evolve to bring a loss or atrophy of muscle tissuse. These diseases are either genetic or accompany hospitalization or prolonged immobilization , or are the result of various diseass. In addition , skeletal muscle gets atrophied during aging and this atrophy is the main cause of fragilty in the elderly . This muscle loss can not be cured today. Various diseases also affect the development and survival of neurons. This is particularly the case of amyotrophic lateral sclerosis (ALS) and spinal muscular atrophy .

    ALS causes progressive paralysis of all skeletal muscles of the limbs and trunk (including respiratory muscles). The cause of ALS is unknown , its incidence increases with age from 40 years and is always fatal . Spinal muscular atrophy is the name given to a group of hereditary diseases characterized by weakness and atrophy of muscles. This disease is transmitted as an autosomal recessive manner. Motor neuron degeneration impairs motor commands to the muscles , resulting in muscle atrophy.

    Projects developed within our team aim to characterize the influence of transcription factors, guidance factors and signaling pathways, in MSC in muscle fiber development and adult, to understand their implications in normal and pathological development of skeletal muscle , and in motor neurons. These studies are based on the use of models of mutant mice for genes of interest , or in cellular models. We hope to get an integrated picture of the response patterns of the muscular system to its environment under normal and pathological conditions through genomic studies. In this context, our current work is organized around four themes.

    • Theme 1 - Embryonic myogenesis

     

    • Theme 2 - Adult Muscle Physiology

     

     

     

     main publications

    • Dos Santos,M., Backer,S., Saintpierre,B., Izac,B., Andrieu,M., Letourneur,F., Relaix,F., Sotiropoulos,A., Maire,P. 2020. Single-nucleus RNA-seq and FISH identify coordinated transcriptional activity in mammalian myofibers. Nat Commun 11, 5102 (2020)
    • Wurmser,M., Chaverot,N., Madani,R , Sakaï,H., Negroni,E., Demignon,J., Saint-Pierre,B., Mouly,V., Amthor,H., Tapscott,S., Birchmeier,C., Tajbakhsh,S., Le Grand,F., Sotiropoulos,A., Maire,P. 2020. SIX1 and SIX4 homeoproteins regulate PAX7+ progenitor cell properties during fetal epaxial myogenesis. 147(19):dev185975. doi: 10.1242/dev.185975.
    • Montel L, Sotiropoulos A, Hénon S. 2019. The nature and intensity of mechanical stimulation drive different dynamics of MRTF-A nuclear redistribution after actin remodeling in myoblasts. PLoS One. 14(3):e0214385. doi: 10.1371/journal.pone.0214385. eCollection 2019.
    • Backer S, Lokmane L, Landragin C, Deck M, Garel S, Bloch-Gallego E. 2018. Trio GEF mediates RhoA activation downstream of Slit2 and coordinates telencephalic wiring. Development. 145(19):dev153692. doi: 10.1242/dev.153692.
    • Randrianarison-Huetz, V. *, Aikaterini Papaefthymiou, A. *, Herledan, G., Noviello, C., Faradova,U., Collard, L., Pincini,A., Schol,E., Decaux, J-F., Maire, P., Vassilopoulos, S., Sotiropoulos, A. 2018. Srf controls satellite cell fusion through the maintenance of actin architecture. J.Cell.Biol. 217(2):685-700