Frontiers in Cell and Developmental Biology (Mar 2022)

Desmin Modulates Muscle Cell Adhesion and Migration

  • Coralie Hakibilen,
  • Florence Delort,
  • Marie-Thérèse Daher,
  • Pierre Joanne,
  • Eva Cabet,
  • Olivier Cardoso,
  • Fany Bourgois-Rocha,
  • Cuixia Tian,
  • Eloy Rivas,
  • Marcos Madruga,
  • Ana Ferreiro,
  • Ana Ferreiro,
  • Alain Lilienbaum,
  • Patrick Vicart,
  • Onnik Agbulut,
  • Sylvie Hénon,
  • Sabrina Batonnet-Pichon

DOI
https://doi.org/10.3389/fcell.2022.783724
Journal volume & issue
Vol. 10

Abstract

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Cellular adhesion and migration are key functions that are disrupted in numerous diseases. We report that desmin, a type-III muscle-specific intermediate filament, is a novel cell adhesion regulator. Expression of p.R406W mutant desmin, identified in patients with desmin-related myopathy, modified focal adhesion area and expression of adhesion-signaling genes in myogenic C2C12 cells. Satellite cells extracted from desmin-knock-out (DesKO) and desmin-knock-in-p.R405W (DesKI-R405W) mice were less adhesive and migrated faster than those from wild-type mice. Moreover, we observed mislocalized and aggregated vinculin, a key component of cell adhesion, in DesKO and DesKI-R405W muscles. Vinculin expression was also increased in desmin-related myopathy patient muscles. Together, our results establish a novel role for desmin in cell-matrix adhesion, an essential process for strength transmission, satellite cell migration and muscle regeneration. Our study links the patho-physiological mechanisms of desminopathies to adhesion/migration defects, and may lead to new cellular targets for novel therapeutic approaches.

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