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Cell Membrane Repair Assay Using a Two-photon Laser Microscope
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Membrane Repair Deficit in Facioscapulohumeral Muscular Dystrophy.

Adam J Bittel1, Sen Chandra Sreetama1, Daniel C Bittel1

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Facioscapulohumeral Muscular Dystrophy (FSHD) myoblasts show impaired plasma membrane repair. Treatments reducing DUX4 expression or oxidative stress improved repair, suggesting new therapeutic targets for FSHD.

Keywords:
DUX4FSHDMOEantioxidantantisense oligonucleotidegapmermembranemusclemyoblastmyofiberrepair

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Area of Science:

  • Muscle biology
  • Cellular repair mechanisms
  • Genetic neuromuscular disorders

Background:

  • Plasma membrane repair deficits are implicated in muscular dystrophies like dysferlinopathy and Duchenne Muscular Dystrophy.
  • Facioscapulohumeral Muscular Dystrophy (FSHD) shares features with other muscular dystrophies, but its plasma membrane repair capacity is uncharacterized.
  • The role of DUX4 expression and oxidative stress in FSHD pathophysiology is increasingly recognized.

Purpose of the Study:

  • To investigate whether human FSHD myoblasts and a murine model of FSHD exhibit plasma membrane repair deficits.
  • To determine the impact of DUX4 expression and oxidative stress on plasma membrane repair in FSHD.
  • To explore potential therapeutic strategies targeting DUX4 and oxidative stress for FSHD.

Main Methods:

  • Focal laser ablation of the sarcolemma was used to assess plasma membrane repair in immortalized human FSHD myoblasts and myofibers from a murine FSHD model (FLExDUX4).
  • Repair kinetics and success were quantified by measuring intracellular FM1-43 dye accumulation post-injury.
  • FSHD myoblasts were treated with a DUX4-targeting antisense oligonucleotide (AON) and the antioxidant Trolox to evaluate their effects on membrane repair.

Main Results:

  • FSHD myoblasts exhibited significantly poorer plasma membrane repair compared to unaffected controls, with a higher percentage of cells failing to repair.
  • Treatment with the DUX4-targeting AON and Trolox ameliorated the observed plasma membrane repair deficits in FSHD myoblasts.
  • Similar plasma membrane repair deficits were observed in myofibers from the murine FLExDUX4 model of FSHD.

Conclusions:

  • This study provides the first evidence of plasma membrane repair deficits in FSHD myoblasts and a murine FSHD model.
  • DUX4 expression and associated oxidative stress appear to play a critical role in the impaired membrane repair observed in FSHD.
  • Targeting DUX4 and oxidative stress presents a promising avenue for developing novel therapeutic interventions for FSHD.