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Phenotypic and Functional Reversion of Muscular Dysgenesis by Heterotypic Fibroblast-Myotube Fusion In Vitro

  • Luis Garcia
  • Patrick Dreyfus
  • Martine Pinçon-Raymond
  • Albert Villageois
  • Olivier Chassande
  • Georges Romey
  • Michel Lazdunski
  • François Rieger
Part of the Advances in Experimental Medicine and Biology book series (AEMB, volume 280)

Abstract

Muscular dysgenesis in the mouse (Gluecksohn-Waelsch 1963; Pai 1965a,b) is a genetic disease with autosomal recessive inheritance characterized by an immature internal organization of foetal muscle (Gluecksohn-Waelsch 1963; Bowden-Essien 1972; Pinçon-Raymond et al., 1985). It is now well established that dysgenic skeletal muscles display important decreases in both the level of the 1,4 dihydropyridine (DHP) receptor (Pinçon-Raymond et al., 1985) and the level of the L-type Ca2+ channel activity (Romey et al., 1986; Beam et al., 1986). These molecular defects have been correlated with the total lack of excitation-contraction coupling in the mutant skeletal muscles (Rieger et al., 1987; Tanabe et al., 1988).

Keywords

Sarcoplasmic Reticulum Contractile Activity Normal Fibroblast Latex Bead Fluorescent Bead 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.

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Copyright information

© Plenum Press, New York 1990

Authors and Affiliations

  • Luis Garcia
    • 1
  • Patrick Dreyfus
    • 1
  • Martine Pinçon-Raymond
    • 1
  • Albert Villageois
    • 1
  • Olivier Chassande
    • 1
  • Georges Romey
    • 2
  • Michel Lazdunski
    • 2
  • François Rieger
    • 1
  1. 1.Pathologie et Régénération du Système Neuromusculaire, INSERM U.153Groupe de DéveloppementParisFrance
  2. 2.Institut de Pharmacologie Moléculaire et Cellulaire UPR 411 CNRSValbonneFrance

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