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Modeling Myotonic Dystrophy 1 in C2C12 Myoblast Cells
09:39

Modeling Myotonic Dystrophy 1 in C2C12 Myoblast Cells

Published on: July 29, 2016

Abnormal prostaglandin E2 production blocks myogenic differentiation in myotonic dystrophy

Daniel Beaulieu1, Philippe Thebault, Richard Pelletier

  • 1Neuroscience Research Unit, Laval University, CHUQ, Pavillon CHUL, Ste-Foy, Quebec, Canada G1V 4G2.

Neurobiology of Disease
|July 12, 2011
PubMed

Insights

Congenital myotonic dystrophy type 1 (DM1) involves muscle cells secreting prostaglandin E2 (PGE2), which hinders muscle development. Inhibiting PGE2 production may restore normal muscle cell differentiation in DM1 patients.

Area of Science:

  • Biochemistry
  • Cell Biology
  • Genetics

Background:

  • Congenital myotonic dystrophy type 1 (DM1) is a severe form of DM1, characterized by CTG repeat expansions and delayed muscle maturation.
  • The underlying mechanisms of congenital DM1 remain largely unknown.

Purpose of the Study:

  • To investigate the molecular mechanisms behind delayed muscle maturation in congenital DM1.
  • To identify soluble factors secreted by DM1 muscle cells that affect myoblast differentiation.

Main Methods:

  • Analysis of muscle satellite cells from congenital DM1 patients with large CTG expansions (>3000 repeats).
  • Identification of secreted factors using cell culture and biochemical assays.
  • Measurement of prostaglandin E2 (PGE2) levels and related enzyme expression (cyclooxygenase 2, mPGES-1).
  • Assessment of intracellular calcium levels and myoblast fusion assays.
  • Testing the effect of acetylsalicylic acid (a COX inhibitor) on DM1 cell differentiation.

Main Results:

  • DM1 muscle satellite cells with large CTG expansions secrete prostaglandin E2 (PGE2), inhibiting normal myoblast fusion.
  • PGE2 production is elevated in DM1 cells due to upregulation of cyclooxygenase 2 (Cox-2), mPGES-1, and prostaglandin EP2/EP4 receptors.
  • High PGE2 levels decrease intracellular calcium, impairing myogenic differentiation.
  • Acetylsalicylic acid treatment normalized PGE2 secretion and restored DM1 muscle cell differentiation.

Conclusions:

  • Delayed muscle maturation in congenital DM1 may stem from an altered autocrine mechanism involving excessive PGE2 secretion.
  • Inhibitors of prostaglandin synthesis show potential for restoring muscle cell differentiation in congenital DM1.

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