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Altered nuclear dynamics in MDX myofibers.

Shama R Iyer1, Sameer B Shah2, Ana P Valencia1

  • 1Department of Orthopaedics, University of Maryland School of Medicine, Baltimore, Maryland.

Journal of Applied Physiology (Bethesda, Md. : 1985)
|December 17, 2016
PubMed
Summary

In Duchenne muscular dystrophy (DMD) mouse models, nuclei move more and transcriptional activity is altered due to disrupted microtubule organization. Fiber malformation contributes more to muscle injury than microtubule structure.

Keywords:
bifurcated fiberscytoskeletonmuscular dystrophy

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Area of Science:

  • Muscle Biology
  • Cellular Biology
  • Biophysics

Background:

  • Duchenne muscular dystrophy (DMD) involves progressive muscle degeneration due to the absence of dystrophin.
  • The precise pathophysiology of dystrophic skeletal muscle, particularly nuclear and cytoskeletal dynamics, remains incompletely understood.

Purpose of the Study:

  • To investigate nuclear movement and the organization of the cytoskeleton in Duchenne muscular dystrophy (DMD) mouse models.
  • To examine the role of the Linkers of Nucleoskeleton and Cytoskeleton (LINC) complex and microtubule architecture in DMD pathophysiology.

Main Methods:

  • Comparative analysis of nuclear movement, LINC complex protein expression, and transcriptional activity in wild-type (WT) and Duchenne muscular dystrophy (MDX) mouse myofibers.
  • Detailed assessment of microtubule density, organization (including 3D core structure), and LINC protein (nesprin-1) expression.
  • Finite element modeling incorporating myofiber morphology and microtubule architecture to assess biomechanical performance.

Main Results:

  • Nuclei in MDX myofibers exhibited significantly increased mobility (distance and velocity) compared to WT myofibers.
  • Reduced expression of nesprin-1 and altered transcriptional activity (histone H3 acetylation) were observed in MDX myofibers.
  • Disrupted microtubule organization was found in both the cortex and core of MDX myofibers; fiber malformation was a greater contributor to biomechanical susceptibility than microtubule structure.

Conclusions:

  • Altered microtubule architecture and LINC complex expression in MDX muscle impact nuclear movement and transcriptional activity.
  • Fiber malformation plays a more significant role than microtubule architecture in the biomechanical vulnerability of dystrophic muscle.
  • These findings provide insights into the cellular mechanisms underlying Duchenne muscular dystrophy and potential therapeutic targets.