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Mice lacking microRNA 133a develop dynamin 2–dependent centronuclear myopathy.

Ning Liu1, Svetlana Bezprozvannaya, John M Shelton

  • 1Department of Molecular Biology and 2Department of Pathology, University of Texas Southwestern Medical Center, Dallas, Texas 75930-9148, USA. Ning.Liu@utsouthwestern.edu

The Journal of Clinical Investigation
|July 9, 2011
PubMed
Summary

Muscle-specific microRNAs, miR-133a-1 and miR-133a-2, are crucial for skeletal muscle health in mice. Their absence causes centronuclear myopathy, impacting muscle function and potentially offering insights into human myopathies.

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Area of Science:

  • Molecular Biology
  • Genetics
  • Muscle Physiology

Background:

  • MicroRNAs (miRNAs) are key regulators of gene expression, influencing cellular phenotypes by targeting messenger RNAs (mRNAs).
  • Skeletal muscle function and homeostasis rely on intricate regulatory networks, including the roles of specific miRNAs.

Purpose of the Study:

  • To investigate the essential roles of muscle-specific microRNAs miR-133a-1 and miR-133a-2 in maintaining adult skeletal muscle structure and function in mice.
  • To explore the link between miR-133a dysregulation and the development of centronuclear myopathy.

Main Methods:

  • Genetic deletion of miR-133a-1 and miR-133a-2 in mice.
  • Analysis of skeletal muscle phenotypes, including myofiber type, mitochondrial function, and muscle triad structure.
  • Investigation of the miR-133a target dynamin 2 mRNA expression.

Main Results:

  • Mice lacking miR-133a-1 and miR-133a-2 developed adult-onset centronuclear myopathy, primarily affecting type II myofibers.
  • Observed abnormalities included impaired mitochondrial function, a shift in myofiber type (fast-to-slow conversion), and disrupted muscle triads.
  • These muscle defects were partly attributed to the dysregulation of dynamin 2 mRNA, a known target of miR-133a and implicated in human centronuclear myopathy.

Conclusions:

  • miR-133a is essential for maintaining adult skeletal muscle integrity, function, bioenergetics, and myofiber identity.
  • Dysregulation of miR-133a contributes to the pathogenesis of centronuclear myopathy.
  • These findings highlight miR-133a as a potential therapeutic target for centronuclear myopathies.